US2018282352A1PendingUtilityA1

Proteasome activity enhancing compounds

64
Assignee: PROTEOSTASIS THERAPEUTICS INCPriority: Nov 12, 2013Filed: Dec 4, 2017Published: Oct 4, 2018
Est. expiryNov 12, 2033(~7.3 yrs left)· nominal 20-yr term from priority
C07B 2200/05C07D 417/14C07D 519/00C07D 209/14C07D 471/04C07D 401/14C07D 401/06C07F 7/1804C07B 59/002C07D 209/12C07D 403/06C07F 7/1856
64
PatentIndex Score
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Claims

Abstract

The present invention is directed to compounds having the Formula (I), (II), (III), (IV), and (V), compositions thereof, and methods for the treatment of a condition associated with a dysfunction in proteostasis.

Claims

exact text as granted — not AI-modified
1 . A compound having the Formula (I): 
       
         
           
           
               
               
           
         
       
       or a pharmaceutically acceptable salt, solvate, clathrate or prodrug thereof; wherein:
 D is selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted aryl, halo, OR c , NR d R d , C(O)OR c , NO 2 , CN, C(O)R c , C(O)C(O)R c , C(O)NR d R d , NR d C(O)R c , NR d S(O) n R c , N(R d )(COOR c ), NR d C(O)C(O)R c , NR d C(O)NR d R d , NR d S(O) n NR d R d , NR d S(O) n R c , S(O) n R c , S(O) n NR d R d , OC(O)OR c , (C═NR d )R c , optionally substituted heterocyclic, and optionally substituted heteroaryl; 
 -A is selected from the group consisting of, ═O, —OH, —C 1 -C 10  alkyl and —C 2 -C 12  alkenyl, wherein the —C 1 -C 10  alkyl and —C 2 -C 12  alkenyl are each optionally substituted; 
 Q 1  is C(R a ) or nitrogen; 
 each of Q 2 , Q 3 , Q 4  and Q 5  is independently selected from the group consisting of C(R 3 ) and nitrogen; wherein at least two of Q 2 , Q 3 , Q 4  and Q 5  are each independently C(R 3 ); 
 each of R i  and R 2  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted aryl, halo, N 3 , OR c , NR d R d , C(O)OR c , NO 2 , CN, C(O)R c , C(O)C(O)R c , C(O)NR d R d , NR d C(O)R c , NR d S(O) n R c , N(R d )(COOR c ), NR d C(O)C(O)R c , NR d C(O)NR d R d , NR d S(O) n NR d R d , NR d S(O) n R c , S(O) n R c , S(O) n NR d R d , OC(O)OR c , (C═NR d )R c , OC(O)R c , optionally substituted heterocyclic, and optionally substituted heteroaryl; 
 each R 3  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted aryl, halo, OR c , N 3 , NR d R d , C(O)OR c , NO 2 , CN, C(O)R c , C(O)C(O)R c , C(O)NR d R d , NR d C(O)R c , NR d S(O) n R c , N(R d )(COOR c ), NR d C(O)C(O)R c , NR d C(O)NR d R d , NR d S(O) n NR d R d , NR d S(O) n R c , S(O) n R c , S(O) n NR d R d , OC(O)OR c , (C═NR d )R c , OC(O)R c , optionally substituted heterocyclic, and optionally substituted heteroaryl; 
 Z is selected from the group consisting of NR b R b , optionally substituted aryl, optionally substituted heterocyclic, optionally substituted heteroaryl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, and OR c ; 
 each R a  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted aryl, halo, N 3 , OR c , NR d R d , C(O)OR c , NO 2 , CN, C(O)R c , C(O)C(O)R c , C(O)NR d R d , NR d C(O)R c , NR d S(O) n R c , N(R d )(COOR c ), NR d C(O)C(O)R c , NR d C(O)NR d R d , NR d S(O) n NR d R d , NR d S(O) n R c , S(O) n R c , S(O) n NR d R d , OC(O)OR c , (C═NR d )R c , OC(O)R c , optionally substituted heterocyclic, and optionally substituted heteroaryl; 
 each R b  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 1 -C 10  alkoxy, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted heterocyclic, optionally substituted aryl, and optionally substituted heteroaryl; 
 each R c  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted heterocyclic, optionally substituted aryl, and optionally substituted heteroaryl; 
 each R d  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 1 -C 10  alkoxy, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted heterocyclic, optionally substituted aryl, and optionally substituted heteroaryl; or two geminal R d  groups are taken together with the nitrogen atom to which they are attached to form an optionally substituted heterocyclic or an optionally substituted heteroaryl; 
 each R e  is independently selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted heterocyclic, optionally substituted aryl, and optionally substituted heteroaryl; and 
 each n is independently 0, 1 or 2; 
 provided that when D is 4-chlorophenyl, -A is ═O, Q 1  is C(R a ), R a  is methyl, Z is 1-piperidinyl, 4-hydroxy-1-piperidinyl or 1-pyrrolidinyl, and each of R i  and R 2  is hydrogen, then at least one of Q 2 , Q 3 , Q 4 , and Q 5  is not CH; and 
 provided that when D is selected from the group consisting of phenyl, 4-chlorophenyl, 4-methylphenyl and 4-methoxyphenyl, -A is ═O, Q 1  is C(R a ), R a  is methyl, Z is 1-piperidinyl or 4-morpholinyl, each of R i  and R 2  is hydrogen, and Q 2  and Q 5  are CH, then Q 3  and Q 4  are not C(OCH 3 ) and C(Br), respectively. 
 
     
     
         2 . The compound of  claim 1 , wherein -A is ═O. 
     
     
         3 . (canceled) 
     
     
         4 . The compound of  claim 1 , wherein R i  and R 2  are each independently selected from hydrogen and optionally substituted C 1 -C 10  alkyl. 
     
     
         5 . (canceled) 
     
     
         6 . (canceled) 
     
     
         7 . The compound of  claim 1 , wherein D is optionally substituted phenyl. 
     
     
         8 . The compound of  claim 1 , wherein Q 1  is C(R a ), and R a  is selected from the group consisting of hydrogen and optionally substituted C 1 -C 10  alkyl. 
     
     
         9 . (canceled) 
     
     
         10 . The compound of  claim 1 , wherein one or two of Q 2 , Q 3 , Q 4  and Q 5  is nitrogen and the remaining of Q 2 , Q 3 , Q 4  and Q 5  are each independently C(R 3 ). 
     
     
         11 - 15 . (canceled) 
     
     
         16 . The compound of  claim 1 , wherein Z is an optionally substituted heterocyclic containing at least one ring nitrogen atom or NR b R b . 
     
     
         17 . The compound of  claim 16 , wherein Z is an optionally substituted N-heterocyclic. 
     
     
         18 . The compound of  claim 16 , wherein Z is selected from the group consisting of optionally substituted pyrrolidinyl and optionally substituted piperidinyl. 
     
     
         19 - 51 . (canceled) 
     
     
         52 . The compound of  claim 1 , wherein the compound has the Formula (IV): 
       
         
           
           
               
               
           
         
       
       wherein:
 R j  is selected from the group consisting of hydrogen, optionally substituted C 1 -C 10  alkyl, optionally substituted C 2 -C 10  alkenyl, optionally substituted C 2 -C 10  alkynyl, optionally substituted C 3 -C 12  cycloalkyl, optionally substituted C 3 -C 12  cycloalkenyl, optionally substituted aryl, halo, N 3 , OR c , NR d R d , C(O)OR c , NO 2 , CN, C(O)R c , C(O)C(O)R c , C(O)NR d R d , NR d C(O)R c , NR d S(O) n R c , N(R d )(COOR c ), NR d C(O)C(O)R c , NR d C(O)NR d R d , NR d S(O) n NR d R d , NR d S(O) n R c , S(O) n R c , S(O) n NR d R d , OC(O)OR c , (C═NR d )R c , OC(O)R c , optionally substituted heterocyclic and optionally substituted heteroaryl. 
 
     
     
         53 . (canceled) 
     
     
         54 . The compound of  claim 52 , wherein R i  and R 2  are each independently selected from hydrogen and optionally substituted C 1 -C 10  alkyl. 
     
     
         55 . (canceled) 
     
     
         56 . The compound of  claim 52 , wherein each of R 3b  and R 3c  is independently selected from the group consisting of hydrogen, halo, NR d R d , NO 2 , CN, optionally substituted C 1 -C 10  alkyl, C(O)OR c , C(O)R c  NR d C(O)R c , OC(O)R c  and OR c . 
     
     
         57 . (canceled) 
     
     
         58 . The compound of  claim 56 , wherein R 3f  is selected from the group consisting of hydrogen, halo, N 3 , C(O)OR c , CN, C(O)R c , C(O)C(O)R c , C(O)NR d R d , NO 2  and NR d R d . 
     
     
         59 . (canceled) 
     
     
         60 . (canceled) 
     
     
         61 . The compound of  claim 52 , wherein R g  and R 3b  are both hydrogen and R 3c  is optionally substituted C 1 -C 6  alkyl, CN and OR c . 
     
     
         62 . (canceled) 
     
     
         63 . The compound of  claim 52 , wherein R g  and R 3c  are both hydrogen and R 3b  is selected from the group consisting of optionally substituted C 1 -C 6  alkyl, CN and OR c . 
     
     
         64 . The compound of  claim 52 , wherein Z is selected from the group consisting of optionally substituted 1-pyrrolidinyl and optionally substituted 1-piperidinyl. 
     
     
         65 - 77 . (canceled) 
     
     
         78 . The compound of  claim 1 , wherein the compound is selected from the group consisting of those shown in the Table below: 
       
         
           
                 
                 
               
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   30 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   31 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   34 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   86 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   87 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   88 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   98 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   104 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   106 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   108 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   115 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   116 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   118 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   119 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   120 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   123 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   125 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   126 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   127 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   128 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   130 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   131 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   132 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   133 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   141 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   142 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   144 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   145 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   146 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   147 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   148 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   149 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   150 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   151 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   152 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   153 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   154 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   155 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   156 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   160 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   161 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   162 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   163 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   164 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   165 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   166 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   167 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   168 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   169 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   170 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   171 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   172 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   173 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   174 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   175 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   176 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   177 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   178 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   179 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   180 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   181 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   182 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   183 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   184 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   185 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   186 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   187 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   188 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   189 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   190 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   191 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   192 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   193 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   194 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   195 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   196 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   197 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   198 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   199 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   200 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   201 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   202 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   203 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   204 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   205 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   206 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   207 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   208 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   209 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   210 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   211 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   212 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   213 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   214 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   215 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   216 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   217 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   218 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   219 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   220 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   230 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   231 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   233 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   234 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   235 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   236 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   237 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   238 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   239 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   240 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   241 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   242 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   243 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   244 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   245 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   246 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   247 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   248 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   249 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   250 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   251 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   252 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   253 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   254 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   255 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   256 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   257 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   258 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   259 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   260 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   261 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   262 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   263 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   264 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   265 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   266 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   267 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   268 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   269 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   270 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   271 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   272 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   273 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   274 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   275 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   276 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   277 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   278 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   279 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   280 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   281 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   282 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   283 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   284 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   285 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   286 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
                   287 
                 
                     
                 
                   
                     
                       
                       
                           
                           
                       
                     
                   
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         79 . A pharmaceutical composition comprising a pharmaceutically acceptable carrier or excipient and a compound of  claim 1 , or a pharmaceutically acceptable salt, solvate, clathrate or prodrug thereof. 
     
     
         80 . (canceled) 
     
     
         81 . (canceled) 
     
     
         82 . A method of treating a patient suffering from a condition associated with a dysfunction in proteostasis comprising administering to said patient an effective amount of a compound of  claim 1 . 
     
     
         83 - 93 . (canceled)

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