Patent ID: 9629816
Date: 2017-04-25
CPC Classifications: A61K,A61P

Claim:
1. A method of preventing, treating or ameliorating a thioesterase deficiency disorder disease selected from neuronal ceroid lipofuscinosis (NCL), infantile NCL, and juvenile NCL, comprising administering to a mammal in need of such treatment, a therapeutically effective amount of a compound that functionally mimics a thioesterase enzymatic activity selected from the group consisting of: N-t-butyl hydroxylamine (NtBUHA), N-Methylhydroxylamine, N,N-Dimethylhydroxylamine, N,N-Diethylhydroxylamine, N-Cyclohexylhydroxylamine, N,O-Bis(trimethylsilyl)hydroxylamine, N-Benzylhydroxylamine, N-Benzyloxycarbonyl), N,O-Di-Boc-hydroxylamine, N-Benzoyl-N-phenyl hydroxylamine, N,N-Dibenzylhydroxylamine, N-tert-Butyl-O-[1-[4-(chloromethyl)phenyl]ethyl]-N-(2-methyl-1-phenylpropyl)hydroxylamine, and pharmaceutically-acceptable salts thereof.