Question of the Day!

Maple Syrup urine disease is due to a defective branched chain alpha-ketoacid dehydrogenase enzyme. A defect in this enzyme causes an inability to metabolize what amino acids?

A) leucine, isoleucine, methionine
B) methionine, valine
C) methionine
D) arginine, lysine, leucine
E) valine, isoleucine, leucine
















Answer: E) valine, isoleucine, leucine. Maple Syrup Urine disease brings a variety of neurological symptoms and a high mortality. It gets its name from the thick, brown urine produced