The Question is from neet-botany biomolecules-biology , and it was created by the author Mr. NARESH KUMAR K

Question

Tay-Sachs disease is caused by a deficiency in the enzyme hexosaminidase A.  This leads to the accumulation of which lipid in the brain?

Mr. NARESH KUMAR K · Accepted Answer

## CORE INFORMATION
✓ **Answer**: C
## CONCEPT FRAMEWORK
 **Primary Concept**: Tay-Sachs disease and its biochemical basis.
 **Related Topics**: Lysosomal storage disorders, sphingolipid metabolism, gangliosides.
 **Prerequisites**: Understanding of enzyme function, lipid metabolism, and the role of lysosomes.
## SOLUTION PATHWAY
**Given Information**:
  Tay-Sachs disease is caused by a deficiency in the enzyme hexosaminidase A.
  The question asks about the lipid accumulating in the brain due to this deficiency.
**Method & Steps**:
 1. Understand the role of hexosaminidase A.
  Reasoning: Hexosaminidase A is a lysosomal enzyme responsible for breaking down gangliosides, a type of glycosphingolipid.
  Formula/Rule:  No specific formula is needed here; this is a conceptual understanding question.
 2. Consider the consequences of hexosaminidase A deficiency.
  Working: If hexosaminidase A is deficient, it cannot break down gangliosides. This leads to their accumulation in cells, particularly in the brain.
  Key Point:  The accumulation of these lipids is what causes the neurological damage characteristic of Tay-Sachs disease.
 3. Identify the specific ganglioside affected.
  Result: Hexosaminidase A specifically breaks down GM2 ganglioside. Therefore, GM2 ganglioside accumulates in the brain in Tay-Sachs disease.
  Verification: This aligns with the established pathophysiology of Tay-Sachs disease.
 ## OPTION ANALYSIS
A :x: Cerebrosides are a type of glycosphingolipid, but they are not the specific substrate of hexosaminidase A.
B :x: Sphingomyelin is another sphingolipid, but its breakdown pathway is different and not directly related to hexosaminidase A deficiency.
C ✓: GM2 ganglioside is the correct answer because it is the substrate of hexosaminidase A, and its accumulation is the hallmark of Tay-Sachs disease.
D :x: Phosphatidylcholine is a phospholipid, not a sphingolipid, and is not directly involved in Tay-Sachs disease pathology.
 ## LEARNING AIDS
 **Common Mistakes**:
  1.Confusing different types of sphingolipids:  Understanding the specific roles of different sphingolipids and their respective enzymes is crucial.
  2.Failing to connect enzyme deficiency to substrate accumulation:  Recognize that enzyme deficiencies result in the buildup of the molecules the enzyme is meant to break down.
 **Quick Tips**:
  1.Focus on the specific enzyme and its substrate in lysosomal storage disorders.
  2.Use mnemonic devices to remember the types of sphingolipids and their associated enzymes.

Mr. NARESH KUMAR K · Answer

Cerebroside

Mr. NARESH KUMAR K · Answer

Sphingomyelin

Mr. NARESH KUMAR K · Answer

The correct Option is "GM2 ganglioside"

Mr. NARESH KUMAR K · Answer

Phosphatidylcholine

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