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Diminished cerebroside-sulfotransferase activity in the Jimpy mouse mutant due to altered lipid composition in
Abstract:
The mouse mutant Jimpy shows a deficient myelination. In the microsomes of the Jimpy brain, the cerebroside-sulfotransferase (EC 2.8.2.11) activity is low. The cerebroside-sulfotransferase activity of Jimpy microsomes could be normalised by delipidating the microsomes with cold acetone and adding to them acetone-extracted lipids from normal microsomes. The lipids extracted from Jimpy membranes did not influence the cerebroside-sulfotransferase activity of neither normal nor Jimpy microsomes. The same results were obtained if artificial cholesterol-phospholipid mixtures in ratios corresponding to the ones found in normal and Jimpy membranes were used for recombination experiments. Therefore the diminished enzyme activities in Jimpy microsomes may be related to the lower cholesterol-phospholipid ratio found in the microsomal membranes of the Jimpy mutant.
Insights
The Jimpy mouse mutant exhibits deficient myelination due to low cerebroside-sulfotransferase activity. This enzyme
Area of Science:
- Neuroscience
- Biochemistry
- Genetics
Background:
- The Jimpy mouse mutant displays impaired myelination, a critical process for nervous system function.
- Microsomal fractions from Jimpy brains show reduced activity of cerebroside-sulfotransferase (EC 2.8.2.11).
Purpose of the Study:
- To investigate the cause of deficient cerebroside-sulfotransferase activity in Jimpy mouse brain microsomes.
- To determine the role of microsomal lipids in the observed enzyme deficiency.
Main Methods:
- Enzyme activity assays were performed on microsomes from normal and Jimpy mice.
- Microsomes were delipidated and reconstituted with lipids from normal or Jimpy sources.
- Lipid composition analysis and recombination experiments with artificial membranes were conducted.
Main Results:
- Delipidation and reconstitution with normal lipids restored cerebroside-sulfotransferase activity in Jimpy microsomes.
- Lipids extracted from Jimpy membranes did not affect enzyme activity in normal or Jimpy microsomes.
- Recombination experiments with artificial cholesterol-phospholipid mixtures mirrored these findings.
Conclusions:
- The reduced cerebroside-sulfotransferase activity in Jimpy microsomes is likely linked to an altered cholesterol-phospholipid ratio in the microsomal membranes.
- This lipid imbalance may underlie the deficient myelination observed in the Jimpy mutant.
