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Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder that presents in newborns, typically within the first 48 hours after birth. As the name suggests, a key indicator of the disease is the presence of a sweet odor in the urine that smells like maple syrup. Left untreated, MSUD can result in failure of central neurological function and the respiratory system and can be fatal. MSUD is caused by mutations in components of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC). These mutations result in the inability for cells to break down branched-chain amino acids (BCAAs). BCAAs and their byproducts accumulate and are excreted in the urine, giving rise to the maple syrup scent.   QUESTIONS: Draw the structures of the three branched-chain amino acids (BCAA). Draw the structures of the three BCAA products released by functional BCKDC. Indicate the original amino acid.

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder that presents in newborns, typically within the first 48 hours after birth. As the name suggests, a key indicator of the disease is the presence of a sweet odor in the urine that smells like maple syrup. Left untreated, MSUD can result in failure of central neurological function and the respiratory system and can be fatal. MSUD is caused by mutations in components of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC). These mutations result in the inability for cells to break down branched-chain amino acids (BCAAs). BCAAs and their byproducts accumulate and are excreted in the urine, giving rise to the maple syrup scent.   QUESTIONS: Draw the structures of the three branched-chain amino acids (BCAA). Draw the structures of the three BCAA products released by functional BCKDC. Indicate the original amino acid.

Biochemistry
Biochemistry
9th Edition
ISBN:9781319114671
Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Chapter1: Biochemistry: An Evolving Science
Section: Chapter Questions
Problem 1P
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Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder that presents in newborns, typically within the first 48 hours after birth. As the name suggests, a key indicator of the disease is the presence of a sweet odor in the urine that smells like maple syrup. Left untreated, MSUD can result in failure of central neurological function and the respiratory system and can be fatal. MSUD is caused by mutations in components of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC). These mutations result in the inability for cells to break down branched-chain amino acids (BCAAs). BCAAs and their byproducts accumulate and are excreted in the urine, giving rise to the maple syrup scent.

 

QUESTIONS:

Draw the structures of the three branched-chain amino acids (BCAA).

Draw the structures of the three BCAA products released by functional BCKDC. Indicate the original amino acid.
 
 
BCKDC PHOSPHATASE BCKDC KINASE S-S E2 LIPOIC ACID DOMAIN E1 DECARBOXYLASE Ela a TPP E13 B E2E1/E3 BINDING DOMAIN E3 DEHYDROGENASE FAD E33 E2 CORE E2 TRANSACYLASE (24 MER)
Transcribed Image Text:BCKDC PHOSPHATASE BCKDC KINASE S-S E2 LIPOIC ACID DOMAIN E1 DECARBOXYLASE Ela a TPP E13 B E2E1/E3 BINDING DOMAIN E3 DEHYDROGENASE FAD E33 E2 CORE E2 TRANSACYLASE (24 MER)
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