Three ATPase activities have an abnormal developmental time course in trembler sciatic nerves

Trembler mutant mice are affected by a peripheral neuropathy characterized by hypomyelination, demyelination, and Schwann cell proliferation. In adult mutants, supernumerary Schwann cells form membranous structures known as 'onion-bulb' formations. The activities of the Na+, K+-ATPase and of two ouabain-insensitive Mg2+-ATPases were investigated in sciatic nerves of young and adult mutants. The Na+, K+-ATPase activities were 92 and 76% of the control values in young and adult mutants, respectively. By immunoblot analysis, the alpha-subunit of the Na+, K+-ATPase had an identical apparent molecular weight in controls at both ages and in young mutants. In adult mutants, on the contrary, the alpha-subunit appeared smaller by about 2 kd, similar to that in kidney, indicating that the Na+, K+-ATPase was localized mainly on supernumerary Schwann cells. In addition, in the mutants, the developmental increase of both the mitochondrial and the nonmitochondrial Mg2+-ATPase was abnormally high. We suggest that the abnormal increase of the nonmitochondrial Mg2+-ATPase activity during development reflects an enrichment of that enzyme in 'onion-bulb' formations