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Physiology · General physiology: peroxisomal function
Sample itemA hypotonic neonate has a high forehead, hepatomegaly and seizures. Plasma shows accumulation of unusual long fatty acids, and liver electron microscopy shows absence of the catalase-containing organelle. Which function is lost?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Chain-shortening of very long-chain fatty acids
Peroxisomes contain catalase and the enzymes that begin beta-oxidation of very long-chain fatty acids, and they take part in plasmalogen synthesis. When peroxisomes fail to form, as in Zellweger syndrome, these fatty acids accumulate and cause neurological and hepatic disease.
Option by option
A. Glycosylation and sorting of secretory proteins
Glycosylation and sorting of secretory proteins occur in the endoplasmic reticulum and Golgi.
B. Oxidation of short-chain fatty acids for ATP
Short-chain fatty acid oxidation occurs in mitochondria, which are present in this child.
C. Chain-shortening of very long-chain fatty acids (correct)
Correct. Peroxisomes shorten very long-chain fatty acids before they enter mitochondria.
D. Hydrolysis of glycolipids at acid pH
Acid hydrolysis of glycolipids is a lysosomal function.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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