title: Increased axonal proteolysis in myelin-deficient mutant mice.
abstract: Protein degradation within retinal ganglion cell axons in vitro is 50 to 110 percent faster than normal in mutant mice exhibiting deficiencies of myelin in the central nervous system. Proteolysis is increased proximally and distally within retinal ganglion cell axons of mice carrying the jumpy mutation or its allele, myelin synthesis deficiency, and is increased distally within those axons of quaking mice. The proteolytic defect is axon (neuron)-specific since the rate of protein degradation within glial cells is normal. Increased axonal proteolysis does not bear a simple relation to hypomyelination since shiverer, another mouse mutant deficient in central myelin, displayed normal rates of axonal protein degradation under the same conditions. These observations suggest an abnormal axon-glial interaction in mice with primary glial defects and raise the possibility that the functioning of histologically normal axons (neurons) may be altered in dysmyelinating diseases.
mesh_terms: D000818:Animals; D001369:Axons / Q000378:metabolism*; D051379:Mice; D008818:Mice, Neurologic Mutants; D009457:Neuroglia / Q000378:metabolism; D009474:Neurons; D011506:Proteins / Q000378:metabolism*; D012160:Retina / Q000166:cytology / Q000378:metabolism*
chemical_list: D011506:Proteins