Enzyme deficient in Wolman disease – Pathology MCQ

The enzyme deficient in Wolman disease is?
A. Alpha galactosidase A
B. Aryl sulphatase A
C. Galactosylceramidase
D. Acid lipase

Correct answer: D. Acid lipase

Autosomal recessiveWolman disease has autosomal recessive inheritance

  • Wolman disease is a lysosomal storage disease caused by deficiency of acid lipase.
  • The major accumulating metabolites are cholesterol esters and triglycerides.

Diseases caused by deficiency of other enzymes:

  • Alpha galactosidase A – Fabry disease
  • Aryl sulphatase A – Metachromatic leukodystrophy
  • Galactosylceramidase – Krabbe disease

Ref: Robbins & Cotran, Pathologic basis of disease, South Asia edition, Vol 1, p151
Image credits: en:User:Cburnett. Licensed under the Creative Commons Attribution-Share Alike 3.0 Unported license. https://en.wikipedia.org/wiki/File:Autorecessive.svg

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