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(Created page with "{{WBRQuestion |QuestionAuthor={{Rim}} |ExamType=USMLE Step 1 |MainCategory=Biochemistry |SubCategory=Neurology |MainCategory=Biochemistry |SubCategory=Neurology |MainCategory=...")
 
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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Biochemistry
|MainCategory=Biochemistry
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|MainCategory=Biochemistry
|MainCategory=Biochemistry
|SubCategory=Neurology
|SubCategory=Neurology
|Prompt=A 6 month old male patient with neurological symptoms is brought by the mother to the physician’s office because she was told her son has an enzyme deficiency.  Upon further work-up, the physician tells the mother that the deficient enzyme is normally responsible to convert pyruvate to acetyl CoA.  The physician instructs the mother that her child is required to be on a ketogenic diet.  Which of the following cofactors is not required for the normal functioning of the enzyme deficient in this patient?
|Prompt=A mother brings her 6-month-old male, with neurological symptoms, to the physician’s office following learning that her son has an enzyme deficiency.  Upon further work-up, you tell the mother that the deficient enzyme is responsible for the conversion of pyruvate to acetyl CoA.  You instruct the mother to place her child on a ketogenic diet.  Which of the following cofactors is not required for the normal function of the enzyme that is deficient in this patient?
|Explanation=[[Pyruvate]] [[dehydrogenase]] [[deficiency]] is characterized by the inability to convert [[pyruvate]] to [[acetyl-CoA]]. Patients with [[pyruvate dehydrogenase deficiency]] are recommended to have [[ketogenic]] diets or diets rich in [[lysine]] and [[leucine]], both of which are considered ketogenic amino acids.  Pyruvate dehydrogenase is contains 3 enzymes that require 5 cofactors: Vitamins B1, B2, B3, B5, and lipoic acid.
|Explanation=[[Pyruvate dehydrogenase deficiency]] is characterized by the inability to convert [[pyruvate]] to [[acetyl-CoA]]. Patients with [[pyruvate dehydrogenase deficiency]] are placed on [[ketogenic]] diets rich in [[lysine]] and [[leucine]], both of which are considered ketogenic amino acids.  Pyruvate dehydrogenase contains 3 enzymes that require 5 cofactors for proper functioning: Vitamins B1, B2, B3, B5, and lipoic acid.  
 
Educational Objective:
Pyruvate dehydrogenase deficiency is characterized by the inability to convert pyruvate to acetyl-CoA. Pyruvate dehydrogenase is a complex that contains 3 enzymes that require 5 cofactors: Vitamins B1, B2, B3, B5, and lipoic acid.


|EducationalObjectives= [[Pyruvate dehydrogenase deficiency]] is characterized by the inability to convert pyruvate to acetyl-CoA. [[Pyruvate dehydrogenase]] contains 3 enzymes that require 5 cofactors for proper functioning: Vitamins B1, B2, B3, B5, and lipoic acid.
|References= First Aid 2014 page 92


|AnswerA=Vitamin B1
|AnswerA=Vitamin B1
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|AnswerEExp=Vitamin B6 is required for the synthesis of cystathionine, heme, GABA, dopamine, niacin, and histamine.  It is not a cofactor of pyruvate dehydrogenase.
|AnswerEExp=Vitamin B6 is required for the synthesis of cystathionine, heme, GABA, dopamine, niacin, and histamine.  It is not a cofactor of pyruvate dehydrogenase.
|RightAnswer=E
|RightAnswer=E
|WBRKeyword=pyruvate, dehydrogenase, complex, vitamin, enzyme, deficiency
|WBRKeyword=pyruvate dehydrogenase, complex, vitamin, vitamins, enzyme, deficiency, cofactors, neurological, acetyl-CoA, pyruvate
|Approved=No
|Approved=Yes
}}
}}

Revision as of 14:35, 21 July 2014
Author [[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Biochemistry
Sub Category SubCategory::Neurology
Prompt [[Prompt::A mother brings her 6-month-old male, with neurological symptoms, to the physician’s office following learning that her son has an enzyme deficiency. Upon further work-up, you tell the mother that the deficient enzyme is responsible for the conversion of pyruvate to acetyl CoA. You instruct the mother to place her child on a ketogenic diet. Which of the following cofactors is not required for the normal function of the enzyme that is deficient in this patient?]]
Answer A AnswerA::Vitamin B1
Answer A Explanation AnswerAExp::Vitamin B1 is a cofactor of pyruvate dehydrogenase.
Answer B AnswerB::Vitamin B2
Answer B Explanation AnswerBExp::Vitamin B2 is a cofactor of pyruvate dehydrogenase.
Answer C AnswerC::Vitamin B3
Answer C Explanation AnswerCExp::Vitamin B3 is a cofactor of pyruvate dehydrogenase.
Answer D AnswerD::Vitamin B5
Answer D Explanation AnswerDExp::Vitamin B5 is a cofactor of pyruvate dehydrogenase.
Answer E AnswerE::Vitamin B6
Answer E Explanation AnswerEExp::Vitamin B6 is required for the synthesis of cystathionine, heme, GABA, dopamine, niacin, and histamine. It is not a cofactor of pyruvate dehydrogenase.
Right Answer RightAnswer::E
Explanation [[Explanation::Pyruvate dehydrogenase deficiency is characterized by the inability to convert pyruvate to acetyl-CoA. Patients with pyruvate dehydrogenase deficiency are placed on ketogenic diets rich in lysine and leucine, both of which are considered ketogenic amino acids. Pyruvate dehydrogenase contains 3 enzymes that require 5 cofactors for proper functioning: Vitamins B1, B2, B3, B5, and lipoic acid.

Educational Objective: Pyruvate dehydrogenase deficiency is characterized by the inability to convert pyruvate to acetyl-CoA. Pyruvate dehydrogenase contains 3 enzymes that require 5 cofactors for proper functioning: Vitamins B1, B2, B3, B5, and lipoic acid.
References: First Aid 2014 page 92]]

Approved Approved::Yes
Keyword WBRKeyword::pyruvate dehydrogenase, WBRKeyword::complex, WBRKeyword::vitamin, WBRKeyword::vitamins, WBRKeyword::enzyme, WBRKeyword::deficiency, WBRKeyword::cofactors, WBRKeyword::neurological, WBRKeyword::acetyl-CoA, WBRKeyword::pyruvate
Linked Question Linked::
Order in Linked Questions LinkedOrder::
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