Q.8 Which of the following cellular organelle is involved in Tay-Sachs disease (TSD)?
1. Golgi
2. Lysosomes
3. Endoplasmic reticulum
4. Mitochondria
Tay-Sachs Disease Organelle: Lysosomes Explained for Biology Exams
Tay-Sachs disease (TSD) is a lysosomal storage disorder caused by deficient hexosaminidase A enzyme activity, leading to GM2 ganglioside buildup in neurons. This MCQ targets biology students preparing for exams like NEET, focusing on cellular organelles in genetic diseases.
Question Breakdown
The query is: “Which cellular organelle is involved in Tay-Sachs disease (TSD)?” Options: 1. Golgi, 2. Lysosomes, 3. Endoplasmic reticulum, 4. Mitochondria.
Correct Answer
2. Lysosomes. TSD results from mutations in the HEXA gene, causing defective β-hexosaminidase A that fails to degrade GM2 gangliosides in lysosomes, leading to their accumulation and neuronal damage.
Option Explanations
Golgi (Option 1)
The Golgi apparatus modifies, sorts, and packages proteins and lipids for secretion or lysosome delivery. In TSD, it processes hexosaminidase A precursors but is not the site of substrate accumulation or primary dysfunction.
Lysosomes (Option 2)
Lysosomes are the main degradative organelles containing acid hydrolases like hexosaminidase A. In TSD, deficient enzyme activity causes undegraded GM2 gangliosides to accumulate, classifying it as a lysosomal storage disorder with progressive neurodegeneration.
Endoplasmic Reticulum (Option 3)
The endoplasmic reticulum (ER) synthesizes and folds hexosaminidase A subunits, including glycosylation and dimerization. Some TSD mutations trigger ER-associated degradation (ERAD) of misfolded enzymes, but the disease pathology stems from lysosomal failure, not ER directly.
Mitochondria (Option 4)
Mitochondria generate ATP and handle oxidative phosphorylation. They play no direct role in TSD, which is a lipid storage issue unrelated to energy production or mitochondrial function.
Organelle Roles Table
| Organelle | Primary Function | TSD Involvement |
|---|---|---|
| Golgi | Protein/lipid modification | Pre-lysosomal processing, not defective |
| Lysosomes | Hydrolytic degradation | GM2 buildup due to enzyme deficiency |
| Endoplasmic Reticulum | Protein synthesis/folding | Mutant enzyme degradation, secondary |
| Mitochondria | Energy production | None |
Lysosomal dysfunction in TSD highlights the organelle’s critical role in cellular waste management, essential for understanding storage disorders in exams.
