Question
Zellweger syndrome is due to the absence of:
| A. |
Lysosome |
| B. |
Mitochondria |
| C. |
Peroxisome |
| D. |
Nucleus |
Show Answer
|
Correct Answer » C Explanation |
 |
- Zellweger syndrome, also called cerebrohepatorenal syndrome, is caused by mutations in PEX (peroxisome biogenesis) genes.
- These mutations impair the formation and function of peroxisomes.
- Consequently, cells have absent or markedly reduced functional peroxisomes.
- This causes accumulation of very-long-chain fatty acids (VLCFAs) and impaired peroxisomal metabolic functions.
- The condition is autosomal recessive.
Why the other options are incorrect
- A. Lysosome — Incorrect
- Lysosomal dysfunction produces lysosomal storage disorders such as Tay-Sachs disease and Gaucher disease.
- B. Mitochondria — Incorrect
- Mitochondrial disorders result from defects in oxidative phosphorylation and mitochondrial metabolism, not Zellweger syndrome.
- D. Nucleus — Incorrect
- Nuclear absence is not the pathological defect in Zellweger syndrome.