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
Wolman 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
