作者: Diwakar S. Deshmukh , Takeshi Inoue , Ronald A. Pieringer
DOI: 10.1016/S0021-9258(18)61861-7
关键词: Metabolism 、 Galactosyltransferase 、 Diglyceride 、 Mutant 、 Central nervous system 、 Microsome 、 Chemistry 、 Biochemistry 、 Myelin 、 Cerebroside
摘要: Abstract The brain of the jimpy mouse, a mutant with severe deficiency myelin in central nervous system, has little ability to synthesize galactosyl diglyceride. Lipase and α- β-galactosidase activity were same as normal littermates. Thus very low concentration monogalactosyl diglyceride found (19 nmoles per g, wet wt) compared (438 must be attributed synthesis by galactosyltransferase pathway rather than overly active degradative enzymes. involved psychosine cerebroside was also reduced brain. small amount that is restricted predominantly microsomal fraction, whereas littermates identical age had both fractions mouse appears unable transfer from microsomes (site synthesis) deposition). relative inability make correlates well absence supports contention diglycerides are associated process myelination.