Patent ID: 8450281

Claim:
A method for treatment of Ullrich congenital muscular dystrophy or Bethlem myopathy in a patient comprising administering to the patient an effective amount of a non-immunosuppressive cyclosporin A derivative of formula I wherein W is MeBmt, dihydro-MeBmt, 8′-hydroxy-MeBmt or O-actetyl-MeBmt, X is αAbu, Val, Thr, Nva or O-methyl threonine (MeOThr), R is Pro, Sar, (D)-MeSer, (D)-MeAla, or (D)-MeSer(Oacetyl), Y is MeLeu, thioMeLeu, v-hydroxy-MeLeu, MeIle, MeVal, MeThr, MeAla, MeaIle or MeaThr; N-ethyl Val (EtVal), N-ethylIle, N-ethylThr, N-ethylPhe, N-ethylTyr or N-ethylThr(Oacetyl), wherein Y cannot be MeLeu when R is Sar, Z is Val, Leu, MeVal or MeLeu, Q is MeLeu, v-hydroxy-MeLeu, MeAla or Pro, T 1 is (D)Ala or Lys, T 2 is MeLeu or γ-hydroxy-MeLeu, and T 3 is MeLeu or MeAla.