When transaminated, the three branched-chain amino acids (valine, leucine, and isoleucine) form compounds that have the characteristic odor of maple syrup. An enzyme known as branched-chain a-keto acid dehydrogenase converts these compounds into CoA esters. People who do not have this enzyme have the genetic disease known as maple syrup urine disease, so called because their urine smells like maple syrup. a. Draw the compounds that smell like maple syrup.b. Draw the CoA esters.c. Branched-chain a-keto acid dehydrogenase has five coenzymes. Identify them.d. Suggest a way to treat maple syrup urine disease.

When transaminated, the three branched-chain amino acids (valine, leucine, and isoleucine) form compounds that have the characteristic odor of maple syrup. An enzyme known as branched-chain a-keto acid dehydrogenase converts these compounds into CoA esters. People who do not have this enzyme have the genetic disease known as maple syrup urine disease, so called because their urine smells like maple syrup. a. Draw the compounds that smell like maple syrup.b. Draw the CoA esters.c. Branched-chain a-keto acid dehydrogenase has five coenzymes. Identify them.d. Suggest a way to treat maple syrup urine disease.

Human Heredity: Principles and Issues (MindTap Course List)

11th Edition

ISBN:9781305251052

Author:Michael Cummings

Publisher:Michael Cummings

Chapter10: From Proteins To Phenotypes

Section: Chapter Questions

Problem 14QP: If phenylalanine was not an essential amino acid, would diet therapy (the elimination of...

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