Question:medium
Zellweger syndrome is due to the absence of which organelle?
Zellweger syndrome is due to the absence of which organelle?
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A peroxisome biogenesis disorder with very long chain fatty acid build-up.
Updated On: Jun 23, 2026
- Lysosome
- Mitochondria
- Peroxisome
- Nucleus
Show Solution
The Correct Option is C
Solution and Explanation
Step 1: Zellweger syndrome belongs to the peroxisome biogenesis disorders, caused by mutations in PEX genes needed to assemble the organelle.
Step 2: The result is a failure to form functional peroxisomes, so the defining feature is reduction or absence of peroxisomes.
Step 3: Without peroxisomal beta-oxidation and plasmalogen production, very long chain fatty acids accumulate and myelin formation suffers, giving the hepatic, renal, and severe neurological features.
Step 4: The other organelles do not fit: lysosomal disease gives storage disorders, mitochondrial disease gives energy-failure syndromes, and a nucleus-less cell cannot survive. \[\boxed{\text{Peroxisome}}\]
Step 2: The result is a failure to form functional peroxisomes, so the defining feature is reduction or absence of peroxisomes.
Step 3: Without peroxisomal beta-oxidation and plasmalogen production, very long chain fatty acids accumulate and myelin formation suffers, giving the hepatic, renal, and severe neurological features.
Step 4: The other organelles do not fit: lysosomal disease gives storage disorders, mitochondrial disease gives energy-failure syndromes, and a nucleus-less cell cannot survive. \[\boxed{\text{Peroxisome}}\]
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