**Question:** Products accumulated in Hunter's syndrome are

A. Glycosaminoglycans
B. Proteoglycans
C. Cholesterol
D. Lipids

**Core Concept:**
Hunter's syndrome, also known as mucopolysaccharidosis II, is a rare and inherited disorder caused by a deficiency in iduronate-2-sulfatase enzyme. This deficiency leads to the accumulation of glycosaminoglycans (GAGs) within cells, particularly in the lysosomes. Glycosaminoglycans are a class of complex carbohydrates that serve various functions, including lubrication, protection, and maintenance of tissue structure.

**Why the Correct Answer is Right:**
In Hunter's syndrome, the deficiency in iduronate-2-sulfatase enzyme impairs the breakdown of glycosaminoglycans, specifically, dermatan sulfate and chondroitin sulfate. As a result, these GAGs accumulate in cells, leading to the clinical manifestations associated with this disorder.

**Why Each Wrong Option is Incorrect:**
A. Proteoglycans are another type of macromolecule containing glycosaminoglycan chains, but they are not accumulated in Hunter's syndrome due to the specific deficiency in iduronate-2-sulfatase enzyme.
B. Cholesterol is not the product accumulated in Hunter's syndrome, as it is not related to the enzymatic deficiency and GAG accumulation.
C. Lipids, while important biomolecules, are not the primary products accumulated in Hunter's syndrome. The primary accumulation is due to the GAGs, specifically glycosaminoglycans.

**Clinical Pearl:**
Hunter's syndrome is one of the mucopolysaccharidoses, a group of lysosomal storage disorders caused by the accumulation of various macromolecules within cells due to deficiencies in lysosomal enzymes. Identifying the specific enzyme deficiency and understanding the accumulation of glycosaminoglycans is crucial for accurate diagnosis and appropriate management of these disorders.