Question:medium
In Zellweger syndrome, there is accumulation of which type of fatty acids?
In Zellweger syndrome, there is accumulation of which type of fatty acids?
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Peroxisomes handle the chains too long for mitochondria.
Updated On: Jun 24, 2026
- Accumulation of long fatty acids
- Accumulation of short chain fatty acids
- Accumulation of very long chain fatty acids
- Accumulation of medium chain fatty acids
Show Solution
The Correct Option is C
Solution and Explanation
Step 1: The key fact is the cellular location of fatty acid breakdown. Mitochondria oxidise short, medium and long chain fatty acids, whereas peroxisomes specialise in trimming the chains that are too long for mitochondria, namely the very long chain fatty acids.
Step 2: Zellweger syndrome is a peroxisome biogenesis disorder. Because functional peroxisomes are absent throughout the body, the one job unique to peroxisomes, oxidation of very long chain fatty acids, cannot be performed.
Step 3: Substrate that cannot be metabolised piles up. Therefore very long chain fatty acids build up in the brain, liver, kidney and muscle, producing the severe neurological and hepatic features and early death seen in affected infants.
Step 4: Since the mitochondrial pathway for short, medium and long chain species is preserved, those fatty acids do not accumulate, ruling out the other choices.
\[\boxed{\text{Accumulation of very long chain fatty acids}}\]
Step 2: Zellweger syndrome is a peroxisome biogenesis disorder. Because functional peroxisomes are absent throughout the body, the one job unique to peroxisomes, oxidation of very long chain fatty acids, cannot be performed.
Step 3: Substrate that cannot be metabolised piles up. Therefore very long chain fatty acids build up in the brain, liver, kidney and muscle, producing the severe neurological and hepatic features and early death seen in affected infants.
Step 4: Since the mitochondrial pathway for short, medium and long chain species is preserved, those fatty acids do not accumulate, ruling out the other choices.
\[\boxed{\text{Accumulation of very long chain fatty acids}}\]
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