US2020148780A1PendingUtilityA1

Cd 14 antagonist antibodies for treating neurodegenerative diseases

Assignee: Implicit Bioscience Pty LtdPriority: Apr 21, 2017Filed: Apr 20, 2018Published: May 14, 2020
Est. expiryApr 21, 2037(~10.7 yrs left)· nominal 20-yr term from priority
C07K 2317/565A61K 45/06C07K 2317/24C07K 16/2896C07K 2317/70C07K 2317/51C07K 2317/515A61P 25/02A61K 38/00A61P 25/28A61K 2039/505A61K 2039/545
42
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Claims

Abstract

This invention relates generally to agents and methods for treating the development or progression of a neurodegenerative disease. In particular, the present invention relates to CD14 antagonists for use in treating the development or progression of a neurodegenerative disease, including Motor Neurone Disease (MND) and Dementia disease or associated symptoms. The present invention further provides compositions including such agents.

Claims

exact text as granted — not AI-modified
What is claimed is: 
     
         1 . A method of inhibiting or decreasing the production of a pro-inflammatory mediator from a mammalian cell in the periphery of a subject with a neurodegenerative disease, comprising, consisting or consisting essentially of contacting membrane bound CD14 (mCD14) or soluble CD14 (sCD14) with a CD14 antagonist antibody in an amount sufficient to inhibit or decrease the production of a pro-inflammatory mediator from a peripheral cell. 
     
     
         2 . A method for treating the development or progression of a neurodegenerative disease or a symptom thereof in a subject, the method comprising, consisting or consisting essentially of administering systemically an effective amount of a CD14 antagonist antibody to the subject. 
     
     
         3 . Use of a CD14 antagonist antibody for treating the development or progression of a neurodegenerative disease, wherein the CD14 antagonist antibody is administered systemically. 
     
     
         4 . The use according to  claim 3 , wherein the CD14 antagonist antibody is manufactured as a medicament for the said application. 
     
     
         5 . The method or use according to any one of  claims 2  to  4 , wherein the CD14 antagonist antibody inhibits or decreases the production of one or more pro-inflammatory mediators by a peripheral cell in the subject. 
     
     
         6 . The method or use according to  claim 5 , wherein the CD14 antagonist antibody binds to both mCD14 and sCD14. 
     
     
         7 . The method or use according to  claim 1 ,  claim 5  or  claim 6 , wherein the peripheral cell is an immune cell. 
     
     
         8 . The method or use according to  claim 7 , wherein the immune cell is selected from a monocyte, macrophage, dendritic cell or T-cell. 
     
     
         9 . The method or use according to any one of  claims 1  to  8 , wherein the pro-inflammatory mediator is a cytokine selected from one or more of tumor necrosis factor-α (TNF-α), interleukin-1 (IL-1)-α, IL-6, IFN-γ, IFN-β, IL-1β, IL-8, IL-17 and IL-18. 
     
     
         10 . The method or use according to any one of  claims 1  to  9 , further comprising identifying that the subject has or is at risk of developing a neurodegenerative disease, suitably prior to administration of the CD14 antagonist antibody. 
     
     
         11 . The method or use according to any one of  claims 1  to  10 , wherein the neurodegenerative disease or disorder is selected from an MND or dementia. 
     
     
         12 . The method or use of  claim 11 , wherein the MND is selected from the group consisting of amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), progressive bulbar palsy (PBP), pseudobulbar palsy. 
     
     
         13 . The method or use according to  claim 12 , wherein the MND is ALS. 
     
     
         14 . The method or use according to  claim 11 , wherein the Dementia is selected from the group consisting of Alzheimer's disease, a Lewy body dementia, Frontotemporal Dementia (FTD) and Vascular Dementia. 
     
     
         15 . The method or use according to anyone of  claims 1  to  14 , wherein the CD14 antagonist antibody is selected from:
 (1) an antibody comprising: 
 a VL domain that comprises, consists or consists essentially of the sequence: 
 
       
         
           
                 
               
                   (3C10 VL) 
                 
                   [SEQ ID NO: 1] 
                 
                   QSPASLAVSLGQRATISCRASESVDSFGNSFMHWYQQKAGQPPKSSIY 
                 
                     
                 
                 
                 
               
                   RAANLESGIPARFSGSGSRTDFTLTINPVEADDVATYFCQQSYEDPWT 
                     
                 
                     
                 
                   FGGGTKLGNQ; 
                     
                 
             
                
                
                
                
               
            
             
                
                
                
               
            
           
         
          and 
         a VH domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (3C10 VH) 
                 
                   [SEQ ID NO: 2] 
                 
                   LVKPGGSLKLSCVASGFTFSSYAMSWVRQTPEKRLEWVASISSGGTT 
                 
                     
                 
                   YYPDNVKGRFTISRDNARNILYLQMSSLRSEDTAMYYCARGYYDYHY 
                 
                     
                 
                   WGQGTTLTVSS; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
         (2) an antibody comprising: 
         a VL domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (28C5 VL) 
                 
                   [SEQ ID NO: 3] 
                 
                   QSPASLAVSLGQRATISCRASESVDSYVNSFLHWYQQKPGQPPKLLI 
                 
                     
                 
                   YRASNLQSGIPARFSGSGSRTDFTLTINPVEADDVATYCCQQSNEDP 
                 
                     
                 
                   TTFGGGTKLEIK; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         a VH domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (28C5 VH) 
                 
                   [SEQ ID NO: 4] 
                 
                   LQQSGPGLVKPSQSLSLTCTVTGYSITSDSAWNWIRQFPGNRLEWMG 
                 
                     
                 
                   YISYSGSTSYNPSLKSRISITRDTSKNQFFLQLNSVTTEDTATYYCV 
                 
                     
                 
                   RGLRFAYWGQGTLVTVSA; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         (3) an antibody comprising: 
         a VL domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (18E12 VL) 
                 
                   [SEQ ID NO: 5] 
                 
                   QTPSSLSASLGDRVTISCRASQDIKNYLNWYQQPGGTVKVLIYYTSR 
                 
                     
                 
                   LHSGVPSRFSGSGSGTDYSLTISNLEQEDFATYFCQRGDTLPWTFGG 
                 
                     
                 
                   GTKLEIK; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         a VH domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (18E12 VH) 
                 
                   [SEQ ID NO: 6] 
                 
                   LESGPGLVAPSQSLSITCTVSGFSLTNYDISWIRQPPGKGLEWLGVI 
                 
                     
                 
                   WTSGGTNYNSAFMSRLSITKDNSESQVFLKMNGLQTDDTGIYYCVRG 
                 
                     
                 
                   DGNFYLYNFDYWGQGTTLTVSS. 
                 
             
                
                
                
                
                
                
                
               
            
           
         
       
     
     
         16 . The method or use according to  claim 15 , wherein the CD14 antagonist antibody comprises, consists or consists essentially of the VL and VH CDR sequences of the above antibodies defined in  claim 13 . 
     
     
         17 . The method or use according to  claim 16 , wherein the CD14 antagonist antibody is selected from:
 (1) an antibody that comprises: a) an antibody VL domain, or antigen binding fragment thereof, comprising L-CDR1, L-CDR2 and L-CDR3, wherein: L-CDR1 comprises the sequence RASESVDSFGNSFMH [SEQ ID NO: 7] (3C10 L-CDR1); L-CDR2 comprises the sequence RAANLES [SEQ ID NO: 8] (3C10 L-CDR2); and L-CDR3 comprises the sequence QQSYEDPWT [SEQ ID NO: 9] (3C10 L-CDR3); and b) an antibody VH domain, or antigen binding fragment thereof, comprising H-CDR1, H-CDR2 and H-CDR3, wherein: H-CDR1 comprises the sequence SYAMS [SEQ ID NO: 10] (3C10 H-CDR1); H-CDR2 comprises the sequence SISSGGTTYYPDNVKG [SEQ ID NO: 11] (3C10 H-CDR2); and H-CDR3 comprises the sequence GYYDYHY [SEQ ID NO: 12] (3C10 H-CDR3);   (2) an antibody that comprises: a) an antibody VL domain, or antigen binding fragment thereof, comprising L-CDR1, L-CDR2 and L-CDR3, wherein: L-CDR1 comprises the sequence RASESVDSYVNSFLH [SEQ ID NO: 13] (28C5 L-CDR1); L-CDR2 comprises the sequence RASNLQS [SEQ ID NO: 14] (28C5 L-CDR2); and L-CDR3 comprises the sequence QQSNEDPTT [SEQ ID NO: 15] (28C5 L-CDR3); and b) an antibody VH domain, or antigen binding fragment thereof, comprising H-CDR1, H-CDR2 and H-CDR3, wherein: H-CDR1 comprises the sequence SDSAWN [SEQ ID NO: 16] (28C5 H-CDR1); H-CDR2 comprises the sequence YISYSGSTSYNPSLKS [SEQ ID NO: 17] (28C5 H-CDR2); and H-CDR3 comprises the sequence GLRFAY [SEQ ID NO: 18] (28C5 H-CDR3); and   (3) an antibody that comprises: a) an antibody VL domain, or antigen binding fragment thereof, comprising L-CDR1, L-CDR2 and L-CDR3, wherein: L-CDR1 comprises the sequence RASQDIKNYLN [SEQ ID NO: 19] (18E12 L-CDR1); L-CDR2 comprises the sequence YTSRLHS [SEQ ID NO: 20] (18E12 L-CDR2); and L-CDR3 comprises the sequence QRGDTLPWT [SEQ ID NO: 21] (18E12 L-CDR3); and b) an antibody VH domain, or antigen binding fragment thereof, comprising H-CDR1, H-CDR2 and H-CDR3, wherein: H-CDR1 comprises the sequence NYDIS [SEQ ID NO: 22] (18E12 H-CDR1); H-CDR2 comprises the sequence VIWTSGGTNYNSAFMS [SEQ ID NO: 23] (18E12 H-CDR2); and H-CDR3 comprises the sequence GDGNFYLYNFDY [SEQ ID NO: 24] (18E12 H-CDR3).   
     
     
         18 . The method or use according to  claim 17 , wherein the CD14 antagonist antibody is humanized. 
     
     
         19 . The method or use according to  claim 18 , wherein the CD14 antagonist antibody comprises a VL domain and a VH domain, wherein:
 the VL domain comprises the amino acid sequence:   
       
         
           
                 
               
                   [SEQ ID NO: 25] 
                 
                   METDTILLWVLLLWVPGSTGDIVLTQSPASLAVSLGQRATISCRASE 
                 
                     
                 
                   SVDSYVNSFLHWYQQKPGQPPKLLIYRASNLQSGIPARFSGSGSRTD 
                 
                     
                 
                   FTLTINPVEADDVATYYCQQSNEDPYTFGGGTKLEIKRTVAAPSVFI 
                 
                     
                 
                   FPPSDEQLKSGTASVVCLLNNFYPREAKVQWKVDNALQSGNSQESVT 
                 
                     
                 
                   EQDSKDSTYSLSSTLTLSKADYEKHKVYACEVTHQGLSSPVTKSFNR 
                 
                     
                 
                   GEC; 
                 
             
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
          and 
         the VH domain comprises the amino acid sequence: 
       
       
         
           
                 
               
                   [SEQ ID NO: 26] 
                 
                   MKVLSLLYLLTAIPGILSDVQLQQSGPGLVKPSQSLSLTCTVTGYSI 
                 
                     
                 
                   TSDSAWNWIRQFPGNRLEWMGYISYSGSTSYNPSLKSRISITRDTSK 
                 
                     
                 
                   NQFFLQLNSVTTEDTATYYCVRGLRFAYWGQGTLVTVSSASTKGPSV 
                 
                     
                 
                   FPLAPCSRSTSESTAALGCLVKDYFPEPVTVSWNSGALTSGVHTFPA 
                 
                     
                 
                   VLQSSGLYSLSSVVTVPSSSLGTKTYTCNVDHKPSNTKVDKRVESKY 
                 
                     
                 
                   GPPCPSCPAPEFLGGPSVFLFPPKPKDTLMISRTPEVTCVVVDVSQE 
                 
                     
                 
                   DPEVQFNWYVDGVEVHNAKTKPREEQFNSTYRVVSVLTVLHQDWLNG 
                 
                     
                 
                   KEYKCKVSNKGLPSSIEKTISKAKGQPREPQVYTLPPSQEEMTKNQV 
                 
                     
                 
                   SLTCLVKGFYPSDIAVEWESNGQPENNYKTTPPVLDSDGSFFLYSRL 
                 
                     
                 
                   TVDKSRWQEGNVFSCSVMHEALHNHYTQKSLSLSLGK. 
                 
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
     
     
         20 . The method or use according to  claim 19 , wherein the CD14 antagonist antibody is IC14, or an antigen-binding fragment thereof. 
     
     
         21 . The method or use according to any one of  claims 1  to  20 , wherein the CD14 antagonist is administered in combination with one or more ancillary anti-neurodegenerative agents that treat the neurodegenerative disease or the symptoms thereof. 
     
     
         22 . The method or use according to  claim 21 , wherein the CD14 antagonist antibody and the ancillary anti-neurodegenerative agent are administered in synergistically effective amounts. 
     
     
         23 . The method or use according to  claim 22 , wherein the CD14 antagonist antibody and the ancillary anti-neurodegenerative agent are administered simultaneously or sequentially. 
     
     
         24 . The method or use according to  claim 23 , wherein the ancillary anti-neurodegenerative agent is an anti-inflammatory. 
     
     
         25 . The method or use according to  claim 24 , wherein the anti-inflammatory is a complement pathway inhibitor. 
     
     
         26 . The method or use according to  claim 25 , wherein the complement pathway inhibitor is a C5a inhibitor. 
     
     
         27 . The method or use according to  claim 26 , wherein the C5a inhibitor is PMX205 or eculizumab. 
     
     
         28 . The method or use according to  claim 23 , wherein the ancillary anti-neurodegenerative agent is an agent that blocks the interaction between CD40 and CD40 ligand. 
     
     
         29 . The method or use according to  claim 28 , wherein the agent is an antibody that bind specifically to CD40 and/or CD40 ligand. 
     
     
         30 . The method or use according to  claim 29 , wherein the antibody is AT-1502. 
     
     
         31 . The method of use according to  claim 20 , wherein the ancillary anti-neurodegenerative agent is riluzole or edaravone. 
     
     
         32 . A pharmaceutical composition for treating the progression or development of a neurodegenerative disease, or a symptom thereof, comprising, consisting or consisting essentially of a CD14 antagonist antibody, together with a pharmaceutically acceptable carrier or diluent, wherein the composition is formulated for systemic administration. 
     
     
         33 . The pharmaceutical composition according to  claim 32 , wherein the CD14 antagonist antibody binds to both sCD14 and mCD14 and inhibits or decreases the production of one or more pro-inflammatory mediators by a peripheral cell in the subject. 
     
     
         34 . The pharmaceutical composition according to  claim 33 , wherein the cell is an immune cell. 
     
     
         35 . The pharmaceutical composition according to  claim 34 , wherein the immune cell is selected from a monocyte, macrophage, dendritic cell or T cell. 
     
     
         36 . The pharmaceutical composition according to any one of  claims 32  to  35 , wherein the pro-inflammatory mediator is a cytokine selected from one or more of tumor necrosis factor-α(TNF-α), interleukin-1 (IL-1)-α, IL-6, IFN-γ, IFN-β, IL-1β, IL-8, IL-17 and IL-18. 
     
     
         37 . The pharmaceutical composition according to any one of  claims 32  to  36 , further comprising identifying that the subject has or is at risk of developing a neurodegenerative disease, suitably prior to administration of the CD14 antagonist antibody. 
     
     
         38 . The pharmaceutical composition according to any one of  claims 32  to  37 , wherein the neurodegenerative disease or disorder is selected from a MND or Dementia. 
     
     
         39 . The pharmaceutical composition of  claim 38 , wherein the MND is selected from the group consisting of amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), progressive bulbar palsy (PBP), pseudobulbar palsy. 
     
     
         40 . The pharmaceutical composition according to  claim 38 , wherein the MND is ALS. 
     
     
         41 . The pharmaceutical composition according to  claim 38 , wherein the dementia is selected from the group consisting of Alzheimer's disease, a Lewy body dementia, Frontotemporal Dementia (FTD) and Vascular Dementia. 
     
     
         42 . The pharmaceutical composition according to anyone of  claims 32  to  41 , wherein the CD14 antagonist antibody is selected from:
 (1) an antibody comprising: 
 a VL domain that comprises, consists or consists essentially of the sequence: 
 
       
         
           
                 
               
                   (3C10 VL) 
                 
                   [SEQ ID NO: 1] 
                 
                   QSPASLAVSLGQRATISCRASESVDSFGNSFMHWYQQKAGQPPKSSI 
                 
                     
                 
                   YRAANLESGIPARFSGSGSRTDFTLTINPVEADDVATYFCQQSYEDP 
                 
                     
                 
                   WTFGGGTKLGNQ; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         a VH domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (3C10 VH) 
                 
                   [SEQ ID NO: 2] 
                 
                   LVKPGGSLKLSCVASGFTFSSYAMSWVRQTPEKRLEWVASISSGGTT 
                 
                     
                 
                   YYPDNVKGRFTISRDNARNILYLQMSSLRSEDTAMYYCARGYYDYHY 
                 
                     
                 
                   WGQGTTLTVSS; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
         (2) an antibody comprising: 
         a VL domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (28C5 VL) 
                 
                   [SEQ ID NO: 3] 
                 
                   QSPASLAVSLGQRATISCRASESVDSYVNSFLHWYQQKPGQPPKLLI 
                 
                     
                 
                   YRASNLQSGIPARFSGSGSRTDFTLTINPVEADDVATYCCQQSNEDP 
                 
                     
                 
                   TTFGGGTKLEIK; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         a VH domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (28C5 VH) 
                 
                   [SEQ ID NO: 4] 
                 
                   LQQSGPGLVKPSQSLSLTCTVTGYSITSDSAWNWIRQFPGNRLEWMG 
                 
                     
                 
                   YISYSGSTSYNPSLKSRISITRDTSKNQFFLQLNSVTTEDTATYYCV 
                 
                     
                 
                   RGLRFAYWGQGTLVTVSA; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         (3) an antibody comprising: 
         a VL domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (18E12 VL) 
                 
                   [SEQ ID NO: 5] 
                 
                   QTPSSLSASLGDRVTISCRASQDIKNYLNWYQQPGGTVKVLIYYTSR 
                 
                     
                 
                   LHSGVPSRFSGSGSGTDYSLTISNLEQEDFATYFCQRGDTLPWTFGG 
                 
                     
                 
                   GTKLEIK; 
                 
             
                
                
                
                
                
                
                
               
            
           
         
          and 
         a VH domain that comprises, consists or consists essentially of the sequence: 
       
       
         
           
                 
               
                   (18E12 VH) 
                 
                   [SEQ ID NO: 6] 
                 
                   LESGPGLVAPSQSLSITCTVSGFSLTNYDISWIRQPPGKGLEWLGVI 
                 
                     
                 
                   WTSGGTNYNSAFMSRLSITKDNSESQVFLKMNGLQTDDTGIYYCVRG 
                 
                     
                 
                   DGNFYLYNFDYWGQGTTLTVSS. 
                 
             
                
                
                
                
                
                
                
               
            
           
         
       
     
     
         43 . The pharmaceutical composition according to  claim 42 , wherein the CD14 antagonist antibody comprises, consists or consists essentially of the VL and VH CDR sequences of the above antibodies defined in  claim 10 . 
     
     
         44 . The pharmaceutical composition according to  claim 43 , wherein the CD14 antagonist antibody is selected from:
 (1) an antibody that comprises: a) an antibody VL domain, or antigen binding fragment thereof, comprising L-CDR1, L-CDR2 and L-CDR3, wherein: L-CDR1 comprises the sequence RASESVDSFGNSFMH [SEQ ID NO: 7] (3C10 L-CDR1); L-CDR2 comprises the sequence RAANLES [SEQ ID NO: 8] (3C10 L-CDR2); and L-CDR3 comprises the sequence QQSYEDPWT [SEQ ID NO: 9] (3C10 L-CDR3); and b) an antibody VH domain, or antigen binding fragment thereof, comprising H-CDR1, H-CDR2 and H-CDR3, wherein: H-CDR1 comprises the sequence SYAMS [SEQ ID NO: 10] (3C10 H-CDR1); H-CDR2 comprises the sequence SISSGGTTYYPDNVKG [SEQ ID NO: 11] (3C10 H-CDR2); and H-CDR3 comprises the sequence GYYDYHY [SEQ ID NO: 12] (3C10 H-CDR3);   (2) an antibody that comprises: a) an antibody VL domain, or antigen binding fragment thereof, comprising L-CDR1, L-CDR2 and L-CDR3, wherein: L-CDR1 comprises the sequence RASESVDSYVNSFLH [SEQ ID NO: 13] (28C5 L-CDR1); L-CDR2 comprises the sequence RASNLQS [SEQ ID NO: 14] (28C5 L-CDR2); and L-CDR3 comprises the sequence QQSNEDPTT [SEQ ID NO: 15] (28C5 L-CDR3); and b) an antibody VH domain, or antigen binding fragment thereof, comprising H-CDR1, H-CDR2 and H-CDR3, wherein: H-CDR1 comprises the sequence SDSAWN [SEQ ID NO: 16] (28C5 H-CDR1); H-CDR2 comprises the sequence YISYSGSTSYNPSLKS [SEQ ID NO: 17] (28C5 H-CDR2); and H-CDR3 comprises the sequence GLRFAY [SEQ ID NO: 18] (28C5 H-CDR3); and   (3) an antibody that comprises: a) an antibody VL domain, or antigen binding fragment thereof, comprising L-CDR1, L-CDR2 and L-CDR3, wherein: L-CDR1 comprises the sequence RASQDIKNYLN [SEQ ID NO: 19] (18E12 L-CDR1); L-CDR2 comprises the sequence YTSRLHS [SEQ ID NO: 20] (18E12 L-CDR2); and L-CDR3 comprises the sequence QRGDTLPWT [SEQ ID NO: 21] (18E12 L-CDR3); and b) an antibody VH domain, or antigen binding fragment thereof, comprising H-CDR1, H-CDR2 and H-CDR3, wherein: H-CDR1 comprises the sequence NYDIS [SEQ ID NO: 22] (18E12 H-CDR1); H-CDR2 comprises the sequence VIWTSGGTNYNSAFMS [SEQ ID NO: 23] (18E12 H-CDR2); and H-CDR3 comprises the sequence GDGNFYLYNFDY [SEQ ID NO: 24] (18E12 H-CDR3).   
     
     
         45 . The pharmaceutical composition according to  claim 44 , wherein the CD14 antagonist antibody is humanized. 
     
     
         46 . The pharmaceutical composition according to  claim 45 , wherein the CD14 antagonist antibody comprises a VL domain and a VH domain, wherein:
 the VL domain comprises the amino acid sequence:   
       
         
           
                 
               
                   [SEQ ID NO: 25] 
                 
                   METDTILLWVLLLWVPGSTGDIVLTQSPASLAVSLGQRATISCRASE 
                 
                     
                 
                   SVDSYVNSFLHWYQQKPGQPPKLLIYRASNLQSGIPARFSGSGSRTD 
                 
                     
                 
                   FTLTINPVEADDVATYYCQQSNEDPYTFGGGTKLEIKRTVAAPSVFI 
                 
                     
                 
                   FPPSDEQLKSGTASVVCLLNNFYPREAKVQWKVDNALQSGNSQESVT 
                 
                     
                 
                   EQDSKDSTYSLSSTLTLSKADYEKHKVYACEVTHQGLSSPVTKSFNR 
                 
                     
                 
                   GEC; 
                 
             
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
          and 
         the VH domain comprises the amino acid sequence: 
       
       
         
           
                 
               
                   [SEQ ID NO: 26] 
                 
                   MKVLSLLYLLTAIPGILSDVQLQQSGPGLVKPSQSLSLTCTVTGYSI 
                 
                     
                 
                   TSDSAWNWIRQFPGNRLEWMGYISYSGSTSYNPSLKSRISITRDTSK 
                 
                     
                 
                   NQFFLQLNSVTTEDTATYYCVRGLRFAYWGQGTLVTVSSASTKGPSV 
                 
                     
                 
                   FPLAPCSRSTSESTAALGCLVKDYFPEPVTVSWNSGALTSGVHTFPA 
                 
                     
                 
                   VLQSSGLYSLSSVVTVPSSSLGTKTYTCNVDHKPSNTKVDKRVESKY 
                 
                     
                 
                   GPPCPSCPAPEFLGGPSVFLFPPKPKDTLMISRTPEVTCVVVDVSQE 
                 
                     
                 
                   DPEVQFNWYVDGVEVHNAKTKPREEQFNSTYRVVSVLTVLHQDWLNG 
                 
                     
                 
                   KEYKCKVSNKGLPSSIEKTISKAKGQPREPQVYTLPPSQEEMTKNQV 
                 
                     
                 
                   SLTCLVKGFYPSDIAVEWESNGQPENNYKTTPPVLDSDGSFFLYSRL 
                 
                     
                 
                   TVDKSRWQEGNVFSCSVMHEALHNHYTQKSLSLSLGK. 
                 
             
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
     
     
         47 . The pharmaceutical composition according to  claim 46 , wherein the CD14 antagonist antibody is IC14, or an antigen-binding fragment thereof. 
     
     
         48 . The pharmaceutical composition according to any one of  claims 31  to  46 , wherein the pharmaceutical composition further comprises an ancillary anti-neurodegenerative agent, wherein the ancillary agent treats the disease or symptoms thereof. 
     
     
         49 . The pharmaceutical composition according to  claim 48 , wherein the ancillary anti-neurodegenerative agent is an anti-inflammatory. 
     
     
         50 . The pharmaceutical composition according to  claim 49 , wherein the anti-inflammatory is a complement inhibitor. 
     
     
         51 . The pharmaceutical composition according to  claim 50 , wherein the complement inhibitor is a C5a inhibitor. 
     
     
         52 . The pharmaceutical composition according to  claim 51 , wherein the C5a inhibitor is PMX205 or eculizumab. 
     
     
         53 . The pharmaceutical composition according to  claim 48 , wherein the ancillary anti-neurodegenerative agent is an agent that blocks the interaction between CD40 and CD40 ligand. 
     
     
         54 . The pharmaceutical composition according to  claim 53 , wherein the agent is an antibody that bind specifically to CD40 and/or CD40 ligand. 
     
     
         55 . The pharmaceutical composition according to  claim 54 , wherein the antibody is AT-1502. 
     
     
         56 . The pharmaceutical composition according to  claim 48 , wherein the ancillary anti-neurodegenerative agent is riluzole or edaravone.

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