Jutzi H, Siegrist H P, Burkart T, Wiesmann U, Herschkowitz N N
Biochim Biophys Acta. 1979 Apr 19;552(3):413-7. doi: 10.1016/0005-2736(79)90185-8.
The mouse mutant Jimpy shows a deficient myelination. In the microsomes of the Jimpy brain, the cerebroside-sulfotransferase (EC 2.8.2.11) activity is low. The cerebroside-sulfotransferase activity of Jimpy microsomes could be normalised by delipidating the microsomes with cold acetone and adding to them acetone-extracted lipids from normal microsomes. The lipids extracted from Jimpy membranes did not influence the cerebroside-sulfotransferase activity of neither normal nor Jimpy microsomes. The same results were obtained if artificial cholesterol-phospholipid mixtures in ratios corresponding to the ones found in normal and Jimpy membranes were used for recombination experiments. Therefore the diminished enzyme activities in Jimpy microsomes may be related to the lower cholesterol-phospholipid ratio found in the microsomal membranes of the Jimpy mutant.
小鼠突变体Jimpy表现出髓鞘形成缺陷。在Jimpy脑的微粒体中,脑苷脂硫酸转移酶(EC 2.8.2.11)活性较低。用冷丙酮使Jimpy微粒体脱脂,并向其中添加从正常微粒体中丙酮提取的脂质,可使Jimpy微粒体的脑苷脂硫酸转移酶活性恢复正常。从Jimpy膜中提取的脂质对正常微粒体和Jimpy微粒体的脑苷脂硫酸转移酶活性均无影响。如果使用比例与正常和Jimpy膜中发现的比例相对应的人工胆固醇 - 磷脂混合物进行重组实验,也会得到相同的结果。因此,Jimpy微粒体中酶活性的降低可能与Jimpy突变体微粒体膜中较低的胆固醇 - 磷脂比例有关。