Kim S, Tuck M, Kim M, Campagnoni A T, Paik W K
Biochem Biophys Res Commun. 1984 Sep 17;123(2):468-74. doi: 10.1016/0006-291x(84)90254-7.
Jimpy mice are dysmyelinating mutants characterized by producing near normal levels of myelin basic protein (MBP) in the brain but failing to incorporate these proteins into the myelin sheath. In this study, the activity of MBP-specific protein-arginine N-methyltransferase (protein methylase I) was studied in the brains of normal and jimpy mice of different ages. The enzyme activity varied little with age in normal mice but in 18 and 21 days-old homozygous jimpy mice the activity was reduced by 50% and 75% respectively from the level of their normal littermates. Interestingly, however, heterozygous jimpy mice who are phenotypically normal and quaking mice (a similar dysmyelinating mutant) showed unaltered enzyme levels.
颤抖小鼠是脱髓鞘突变体,其特征是大脑中髓鞘碱性蛋白(MBP)的产生水平接近正常,但无法将这些蛋白质整合到髓鞘中。在本研究中,研究了不同年龄的正常小鼠和颤抖小鼠大脑中MBP特异性蛋白精氨酸N-甲基转移酶(蛋白甲基化酶I)的活性。正常小鼠的酶活性随年龄变化不大,但在18日龄和21日龄的纯合颤抖小鼠中,其活性分别比正常同窝小鼠降低了50%和75%。然而,有趣的是,表型正常的杂合颤抖小鼠和震颤小鼠(一种类似的脱髓鞘突变体)的酶水平未发生改变。