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MBBS QUESTION #10351
Question 1
Long-chain fatty acids must be transported into the mitochondrial matrix for beta-oxidation via a specific carrier system, impaired in carnitine palmitoyltransferase (CPT) deficiency. This transport system is known as the:
  • Carnitine shuttle✔️
  • Malate-aspartate shuttle
  • Glycerol-3-phosphate shuttle
  • Citrate shuttle
Correct Answer Explanation
The carnitine shuttle — comprising CPT-I (outer mitochondrial membrane), carnitine-acylcarnitine translocase, and CPT-II (inner membrane) — converts long-chain fatty acyl-CoA to acylcarnitine for transport into the mitochondrial matrix, where it is reconverted to acyl-CoA for beta-oxidation; the malate-aspartate and glycerol-3-phosphate shuttles instead transport reducing equivalents (NADH) rather than fatty acids.