## **Core Concept**
Glucosylceramide is a type of glycosphingolipid that accumulates in certain lysosomal storage disorders. The question tests the knowledge of specific lipid storage diseases and their associated accumulated substances.
## **Why the Correct Answer is Right**
Glucosylceramide accumulation is characteristic of Gaucher's disease, which is caused by a deficiency of the enzyme **glucocerebrosidase**. This enzyme is necessary for breaking down glucosylceramide into glucose and ceramide. Without it, glucosylceramide accumulates within cells, particularly in macrophages, leading to various clinical manifestations.
## **Why Each Wrong Option is Incorrect**
- **Option A:** This option is incorrect because it does not correspond with the accumulation of glucosylceramide.
- **Option B:** This option is incorrect as it relates to a different condition or substance.
- **Option C:** This option might relate to other types of lipid storage diseases but is not associated with glucosylceramide accumulation.
- **Option D:** This option is incorrect because it does not correctly identify the condition associated with glucosylceramide accumulation.
## **Clinical Pearl / High-Yield Fact**
A key clinical pearl is that Gaucher's disease is treated with **enzyme replacement therapy (ERT)** using imiglucerase, which is a recombinant form of the glucocerebrosidase enzyme. This treatment helps to reduce the accumulation of glucosylceramide.
## **Correct Answer:** . **Gaucher's disease**
Free Medical MCQs Β· NEET PG Β· USMLE Β· AIIMS
Access thousands of free MCQs, ebooks and daily exams.
By signing in you agree to our Privacy Policy.