X-linked adrenoleukodystrophy (X-ALD) is caused by a defect in peroxisomal impor

Question

X-linked adrenoleukodystrophy (X-ALD) is caused by a defect in peroxisomal import of very-long-chain saturated fatty acids. Why is this harmful to the body?

Choose one:

A. Very-long-chain fatty acids are not substrates for mitochondrial ? oxidation.

B. Very-long-chain fatty acids are required for peroxisomal function.

C. Blocking peroxisomal import leads to product inhibition of cytosolic acyl-CoA synthetases.

D. Inhibition of peroxisomal import reduces the amount of ATP that can be synthesized.

Explanation / Answer

C. Blocking peroxisomal import leads to product inhibition of cytosolic acyl-CoA synthetases.

X-ALD is happened due to mutation in ABCD1 gene. ABCD1 gene encode a protein of ABC transporter family. These proteins are required to transpost long chain fatty acids to peroxisome where they are degraded by beta oxidation.During beta oxidation in the cytoplasm fatty acids are broken to produce acetyl-CoA which next passed to citric acid cycle.

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