Full 2L QBank
Biochemistry General 7456e681

Beta galactosidase deficiency causes?

A
Goucher disease
B
Krabbe's disease
C
Fabry's disease
D
Neimann Pick disease
High-Yield Explanation
Ans. is 'b' i.e., Krabbe's disease Krabbe disease, also known as globoid cell leukodystrophy or galactosylceramide lipidosis, is an autosomal-recessive sphingolipidosis caused by deficient activity of the lysosomal hydrolase galactosylceramide beta-galactosidase (GALC).GALC degrades galactosylceramide, a major component of myelin, and other terminal beta-galactose-containing sphingolipids, including psychosine (galactosylsphingosine).Beta-galactosidase is a lysosomal enzyme responsible for catalyzing the hydrolysis of gangliosides. The deficiency of this enzyme can lead to 1 of the following conditions: GM1 gangliosidosis, Morquio syndrome B, and galactosialidosis.

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