Decarboxylation of valine, leucine and isolucine is defective in which of the following conditions?
Branched chain keto acids include valine, leucine and isoleucine. Leucine is ketogenic, valine is glucogenic and isoleucine is both glucogenic and ketogenic. The thiamine requiring enzymes required for decarboxylation of these aminoacids are defective in maple syrup urine disease (MSUD). Ref: Textbook of Biochemistry By DM Vasudevan, 3rd Edition, Page 160.
Maple Syrup urine disease
Hanup disease
Alkaptonuria
GM1 Gangliosidosis