In Gaucher’s disease there is accumulation of —inside the cells –
**Core Concept**
Gaucher's disease is a genetic disorder caused by a deficiency of the enzyme glucocerebrosidase, which leads to the accumulation of a particular lipid within cells.
**Why the Correct Answer is Right**
In Gaucher's disease, the deficiency of glucocerebrosidase results in the inability to break down glucocerebroside, a type of glycosphingolipid. This accumulation of glucocerebroside within cells leads to their dysfunction and eventual cell death. The glucocerebroside accumulates in the lysosomes, causing them to become engorged and leading to cellular damage.
**Why Each Wrong Option is Incorrect**
**Option A:** None of the other options accurately describe the lipid accumulation in Gaucher's disease.
**Option B:** Sphingomyelin is the lipid that accumulates in Niemann-Pick disease, not Gaucher's disease.
**Option C:** Ceramide is a type of sphingolipid, but it is not the lipid that accumulates in Gaucher's disease.
**Clinical Pearl / High-Yield Fact**
It's essential to remember that Gaucher's disease is an autosomal recessive disorder, meaning that a person must inherit two defective copies of the GBA gene (one from each parent) to develop the disease.
**Correct Answer: D. Glucocerebroside.**