Use of Organic Compound for the Treatment of Noonan Syndrome

Abstract

The use of a MEK inhibitor compound of Formula (I), as defined herein, or a pharmaceutically acceptable salt thereof for the preparation of a medicament for the treatment of Noonan Syndrome, a method of treating a warm-blooded animal, especially a human, having Noonan Syndrome, comprising administering to said animal a therapeutically effective amount of a MEK inhibitor compound of Formula (I), as defined herein, or a pharmaceutically acceptable salt thereof, and to a pharmaceutical composition and a commercial package comprising a compound of Formula (I) or a pharmaceutically acceptable salt thereof, and a package insert or other labeling including directions for treating Noonan Syndrome.

1 . (canceled) 
     
     
         2 . A method for treating hypertrophic cardiomyopathy in a patient in need thereof, the method comprising administering to the patient a therapeutically effective amount of a compound of Formula (I): 
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         3 . The method of  claim 2 , wherein the hypertrophic cardiomyopathy is neonatal hypertrophic cardiomyopathy. 
     
     
         4 . The method of  claim 2 , wherein the hypertrophic cardiomyopathy is associated with defects in the Ras-Raf-Mek-Erk pathway. 
     
     
         5 . The method of  claim 2 , wherein the hypertrophic cardiomyopathy is associated with overexpression of a MEK kinase. 
     
     
         6 . The method of  claim 5 , wherein the MEK kinase is selected from the group consisting of MEK1, MEK2, and combinations thereof. 
     
     
         7 . A method for treating hypertrophic cardiomyopathy in a patient in need thereof, the method comprising:
 (a) determining if the hypertrophic cardiomyopathy is associated with overexpression of a MEK kinase; and   (b) if the hypertrophic cardiomyopathy is associated with overexpression of a MEK kinase, administering to the patient a therapeutically effective amount of a compound of Formula (I):   
       
         
           
           
               
               
           
         
         or a pharmaceutically acceptable salt thereof. 
       
     
     
         8 . The method of  claim 7 , wherein the hypertrophic cardiomyopathy is neonatal hypertrophic cardiomyopathy. 
     
     
         9 . The method of  claim 7 , wherein the hypertrophic cardiomyopathy is associated with defects in the Ras-Raf-Mek-Erk pathway. 
     
     
         10 . The method of  claim 7 , wherein the MEK kinase is selected from the group consisting of MEK1, MEK2, and combinations thereof. 
     
     
         11 . The method of  claim 9 , wherein the MEK kinase is selected from the group consisting of MEK1, MEK2, and combinations thereof.