What product will accumulate if the there is a degradation defect involving the given amino acids? Choices: ?-ketoisovalerate ?-keto-β-methylvalerate ?-ketoisocaproate Phenylpyruvate 4-hydroxyphenylpyruvate dioxygenase   Isoleucine Phenylalanine Leucine Valine Tyrosine

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
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What product will accumulate if the there is a degradation defect involving the given amino acids?

Choices:

?-ketoisovalerate

?-keto-β-methylvalerate

?-ketoisocaproate

Phenylpyruvate

4-hydroxyphenylpyruvate dioxygenase

 

  1. Isoleucine

  2. Phenylalanine

  3. Leucine

  4. Valine

  5. Tyrosine



Amino Acid Degradation Pathway (some choices can be selected more than once)

Choices:

Phenylalanine

Tyrosine

Cystine

Cysteine

Branched-Chain Amino Acids

Aromatic Amino Acids

 

  1. Richner-Hanhart Syndrome

  2. Tyrosinemia Type III

  3. Cystinuria

  4. Maple-Syrup Urine Disease

  5. Tyrosinemia Type 1

  6. Phenylketonuria

  7. Black-Urine Disease

 

Management (some choices may be selected more than once). The number after the condition represents the number of answers required for that item.

Choices:

Sapropterin

Pegvaliase

Methionine restriction

Vitamin A derivatives

Emollients

Nitisinone

No treatment yet

Vitamin B1

Vitamin C

Acetazolamide,

Hyperdiuresis

L-CDME

 

  1. Phenylketonuria (2)

  2. Cystinuria (3)

  3. Black Urine Disease (2)

  4. Richner-Hanhart Syndrome (3)

  5. Tyrosinemia Type III (1)

  6. Maple-Syrup Urine (1)

  7. Tyrosinemia Type I (1)

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