Question

Krabbe’s disease is:

• A. Deficiency of Beta- galactosylcermidase
• B. Deficiency of aryl sulphatase
• C. Alpha galactosidase
• D. Acid lipase

Answer 1

The Correct Option is A

Krabbe’s disease, also known as Globoid Cell Leukodystrophy, is a rare genetic disorder that affects the nervous system. It is caused by a deficiency of an enzyme known as beta-galactosylcermidase (also called galactocerebrosidase). This enzyme deficiency leads to the accumulation of certain lipids in the cells, which ultimately harms the nervous system and causes severe deterioration.

The key points to understand regarding Krabbe’s disease are:

• It is a lysosomal storage disorder.
• The main enzyme deficiency involved is beta-galactosylcermidase.
• It results in severe neurological symptoms due to the build-up of galactolipids.
• Early diagnosis is crucial for managing the disease effectively.

In summary, the correct answer to the question "Krabbe’s disease is:" is a deficiency of Beta-galactosylcermidase.