Biochemistry, The Molecular Basis of Life, 6th Edition
6th Edition
ISBN: 9780190259204
Author: Trudy McKee, James R. McKee
Publisher: Oxford University Press
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Question
Chapter 9, Problem 55TQ
Summary Introduction
To review:
The way in which pyruvate deficiency causes elevated blood levels of lactate, pyruvat, end alanine.
Introduction:
Pyruvate dehydrogenase deficiency (PDH) is an X-linked disorder generally caused by a mutation in the genes encoding PDH subunits. PDH deficiency is characterized by mental retardation, lactic acidosis, coma, and microcephaly. It is usually diagnosed in children more than in adults.
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Pyruvate is accepted into the TCA cycle by a “feeder” reaction using the pyruvatedehydrogenase complex, resulting in acetyl-CoA and CO2. Provide a full mechanismfor this reaction utilizing the TPP cofactor. Include the roles of all cofactors.
B- Vitamins are converted readily into important metabolic cofactors. Deficiency inany one of them has serious side effects.
a. The disease beriberi results from a vitamin B 1 (Thiamine) deficiency and ischaracterized by cardiac and neurological symptoms. One key diagnostic forthis disease is an increased level of pyruvate and α-ketoglutarate in thebloodstream. How does this vitamin deficiency lead to increased serumlevels of these factors?
b. What would you expect the effect on the TCA intermediates for a patientsuffering from vitamin B 5 deficiency?
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Draw the Krebs Cycle and show the entry points for the amino acids Alanine,Glutamic Acid, Asparagine, and Valine into the Krebs Cycle - (Draw the Mechanism).
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Chapter 9 Solutions
Biochemistry, The Molecular Basis of Life, 6th Edition
Ch. 9 - Prob. 1QCh. 9 - Prob. 2QCh. 9 - Prob. 3QCh. 9 - Prob. 4QCh. 9 - Prob. 5QCh. 9 - Prob. 6QCh. 9 - Prob. 7QCh. 9 - Prob. 1RQCh. 9 - Prob. 2RQCh. 9 - Prob. 3RQ
Ch. 9 - Prob. 4RQCh. 9 - Prob. 5RQCh. 9 - Prob. 6RQCh. 9 - Prob. 7RQCh. 9 - Prob. 8RQCh. 9 - Prob. 9RQCh. 9 - Prob. 10RQCh. 9 - Prob. 11RQCh. 9 - Prob. 12RQCh. 9 - Prob. 13RQCh. 9 - Prob. 14RQCh. 9 - Prob. 15RQCh. 9 - Prob. 16RQCh. 9 - Prob. 17RQCh. 9 - Prob. 18RQCh. 9 - Prob. 19RQCh. 9 - Prob. 20RQCh. 9 - Prob. 21RQCh. 9 - Prob. 22RQCh. 9 - Prob. 23RQCh. 9 - Prob. 24RQCh. 9 - Prob. 25RQCh. 9 - Prob. 26RQCh. 9 - Prob. 27RQCh. 9 - Prob. 28FBCh. 9 - Prob. 29FBCh. 9 - Prob. 30FBCh. 9 - Prob. 31FBCh. 9 - Prob. 32FBCh. 9 - Prob. 33FBCh. 9 - Prob. 34FBCh. 9 - Prob. 35FBCh. 9 - Prob. 36FBCh. 9 - Prob. 37FBCh. 9 - Prob. 38SACh. 9 - Prob. 39SACh. 9 - Prob. 40SACh. 9 - Prob. 41SACh. 9 - Prob. 42SACh. 9 - Prob. 43TQCh. 9 - Prob. 44TQCh. 9 - Prob. 45TQCh. 9 - Prob. 46TQCh. 9 - Prob. 47TQCh. 9 - Prob. 48TQCh. 9 - Prob. 49TQCh. 9 - Prob. 50TQCh. 9 - Prob. 51TQCh. 9 - Prob. 52TQCh. 9 - Prob. 53TQCh. 9 - Prob. 54TQCh. 9 - Prob. 55TQCh. 9 - Prob. 56TQCh. 9 - Prob. 57TQCh. 9 - Prob. 58TQCh. 9 - Prob. 59TQ
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