Medicine & USMLE

Adrenoleukodystrophy

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Peroxisome
  1. Zellweger Syndrome
  2. Adrenoleukodystrophy
  3. Refsum Disease

Adrenoleukodystrophy is an X-linked recessive disorder, and it is the most common peroxisome disease.

It is caused by a mutation in ABCD1, which impairs the transport of very long chain fatty acids (VLCFAs) into the peroxisome. This in turn impairs beta-oxidation of these fatty acids, and causes their accumulation.

The most severely affected tissues are the CNS white matter (causing neurologic defects), the adrenal cortex (causing adrenal insufficiency), and the Leydig cells in the testes (causing hypogonadism).

Find this Adrenoleukodystrophy mnemonic and more mnemonics for Peroxisome among Pixorize's visual mnemonics for the USMLE Step 1 and NBME Shelf Exams.