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Three ATPase activities have an abnormal developmental time course in trembler sciatic nerves
Developmental Neuroscience
|January 1, 1987
Summary
Trembler mutant mice exhibit peripheral neuropathy with altered ATPase activities in sciatic nerves. Abnormalities in sodium-potassium ATPase and magnesium-ATPases suggest enzyme redistribution within Schwann cells and onion-bulb formations.
Area of Science:
- Neuroscience
- Biochemistry
- Genetics
Background:
- Trembler mutant mice display peripheral neuropathy, including hypomyelination, demyelination, and Schwann cell proliferation.
- Adult mutants develop characteristic 'onion-bulb' formations composed of supernumerary Schwann cells.
Purpose of the Study:
- To investigate the activities of Na+, K+-ATPase and Mg2+-ATPases in the sciatic nerves of young and adult Trembler mutant mice.
- To determine the localization of Na+, K+-ATPase alpha-subunit in adult mutant sciatic nerves.
Main Methods:
- Enzyme activity assays for Na+, K+-ATPase and Mg2+-ATPases.
- Immunoblot analysis to assess the molecular weight and localization of the Na+, K+-ATPase alpha-subunit.
Main Results:
- Na+, K+-ATPase activities were reduced in both young (92%) and adult (76%) mutants compared to controls.
- Immunoblot analysis revealed a smaller apparent molecular weight of the Na+, K+-ATPase alpha-subunit in adult mutants, suggesting its localization on supernumerary Schwann cells.
- Developmental increases in both mitochondrial and non-mitochondrial Mg2+-ATPase activities were abnormally high in mutants.
Conclusions:
- The findings suggest altered Na+, K+-ATPase localization in adult Trembler mutants, potentially on supernumerary Schwann cells within onion-bulb formations.
- The abnormal increase in Mg2+-ATPase activity may indicate enrichment of this enzyme in onion-bulb structures, reflecting adaptive or pathological changes in Schwann cells.