Difficulty: Easy
Correct Answer: Acyl-carnitine
Explanation:
Introduction / Context:
Long-chain fatty acids require specialized transport to enter the mitochondrial matrix for beta-oxidation. The carnitine shuttle ensures regulated import and prevents futile cycles with fatty acid synthesis.
Given Data / Assumptions:
Concept / Approach:
Carnitine palmitoyltransferase I (CPT I) on the outer membrane forms acyl-carnitine from acyl-CoA and carnitine. A translocase shuttles acyl-carnitine across the inner membrane. In the matrix, CPT II regenerates acyl-CoA and releases carnitine to return to the cytosolic side. Malonyl-CoA inhibits CPT I to coordinate oxidation with synthesis.
Step-by-Step Solution:
Activate fatty acid: FA + CoA + ATP → acyl-CoA + AMP + PPi.CPT I reaction: acyl-CoA + carnitine → acyl-carnitine + CoA.Translocase moves acyl-carnitine into matrix.CPT II reaction: acyl-carnitine + CoA → acyl-CoA (matrix) + carnitine.
Verification / Alternative check:
Inborn errors of CPT I/II or carnitine deficiency impair beta-oxidation, causing hypoketotic hypoglycemia and muscle weakness; supplementation and dietary management target this shuttle.
Why Other Options Are Wrong:
Common Pitfalls:
Confusing activation (acyl-CoA formation) with transport (acyl-carnitine intermediate), and overlooking malonyl-CoA’s inhibitory role.
Final Answer:
Acyl-carnitine.
Discussion & Comments