Gaucher’s disease is due to deficiency of enzyme
First, I need to recall which enzyme is involved in Gaucher's. I think it's glucocerebrosidase. Wait, is that right? Let me double-check. Glucocerebrosidase breaks down glucocerebroside into glucose and ceramide. If that enzyme is deficient, glucocerebroside can't be broken down, leading to accumulation in macrophages, which become Gaucher cells. That sounds familiar from my studies.
Now, looking at the options, the correct answer should be glucocerebrosidase. Let's see the other options. Option A might be something like alpha-galactosidase, which is involved in Fabry disease. Option B could be beta-glucosidase, but wait, beta-glucosidase is actually another name for glucocerebrosidase? Or is there a distinction? Maybe the options are using different names. Another possibility is acid sphingomyelinase, which is involved in Niemann-Pick disease. So if the options include that, then it's incorrect.
So the key here is to remember that Gaucher's is due to glucocerebrosidase deficiency. The other options are for different diseases. For example, Tay-Sachs is due to hexosaminidase A deficiency. Niemann-Pick is acid sphingomyelinase. Fabry is alpha-galactosidase. So each of these enzymes is linked to specific diseases.
I need to make sure that the correct answer is glucocerebrosidase. Let me confirm. Yes, Gaucher's disease is indeed caused by a deficiency in beta-glucocerebrosidase, also known as glucosylceramidase. The accumulation of glucocerebroside in macrophages leads to the characteristic Gaucher cells, which are large, have a crumpled paper appearance, and are found in the spleen, liver, and bone marrow.
So the correct answer is the enzyme that breaks down glucocerebroside. The other options would be for different diseases. For example, if one option is beta-glucosidase, that's the same enzyme. If another option is alpha-glucosidase, that's involved in Pompe disease. So the key is to match the enzyme to the disease correctly.
**Core Concept**
Gaucher’s disease is a lysosomal storage disorder caused by deficiency of **beta-glucocerebrosidase (glucosylceramidase)**, leading to accumulation of **glucocerebroside** in macrophages. This enzyme deficiency disrupts sphingolipid metabolism, resulting in characteristic **Gaucher cells** in reticuloendothelial tissues.
**Why the Correct Answer is Right**
Beta-glucocerebrosidase catalyzes the hydrolysis of glucocerebroside into glucose and ceramide. Its deficiency causes undigested glucocerebroside to accumulate in lysosomes of macrophages, forming **Gaucher cells** (abund