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Physiology · Nerve and muscle: carnitine shuttle in muscle
Sample itemA 22-year-old has muscle pain and dark urine after prolonged exercise during a fast. Venous lactate rises normally during ischaemic forearm exercise, and glycogen stores are normal. Which process is deficient?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Carnitine-mediated entry of long-chain fatty acids into mitochondria
Long-chain fatty acids need carnitine palmitoyltransferase to enter mitochondria, and this is the main fuel in prolonged exercise and fasting. Deficiency of CPT II causes exertional rhabdomyolysis with normal glycolysis, so lactate rises normally. In glycogen phosphorylase deficiency, lactate fails to rise.
Option by option
A. Breakdown of glycogen by muscle phosphorylase
A phosphorylase defect would prevent the lactate rise in the forearm test.
B. Carnitine-mediated entry of long-chain fatty acids into mitochondria (correct)
Correct. Impaired fatty acid entry causes problems in prolonged exercise or fasting with intact glycolysis.
C. Pyruvate conversion to lactate in the cytosol
Lactate production was normal.
D. Oxidation of ketone bodies in cardiac muscle
Ketone oxidation in the heart is not the cause of this muscle presentation.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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