Ganser A L, Kerner A L, Brown B J, Davisson M T, Kirschner D A
Department of Neuroscience, Children's Hospital, Boston, Mass.
Dev Neurosci. 1988;10(2):123-40. doi: 10.1159/000111963.
The lipids of white matter and peripheral nerve from neurological mutant mice with possible myelin abnormalities were analyzed by thin-layer chromatography and quantitated by densitometry. Eight mutants had major abnormalities in the central nervous system (CNS) and/or peripheral nervous system (PNS) tissues examined (optic nerve, and trigeminal and sciatic nerves). In the optic nerve of axJ/axJ, there were increases of 20-30% in the levels of the major phospholipids; peripheral nerve was normal. In bc3J/bc3J CNS, the major phospholipids and cholesterol were increased by 25-40%; the PNS was normal. In myd/myd CNS, there were increases of about 20% in the levels of both forms of cerebrosides and in the major phospholipids; in the PNS the lipids were normal. ot/ot CNS had 20-40% reductions of all the glycolipids and minor alterations in some of the phospholipids and cholesterol; the PNS had 20% losses of both forms of cerebrosides. In the PNS of ji/ji, there were decreases of 10-40% among the glycolipids and of 15-25% in three of the major phospholipids; the CNS was virtually normal. In the PNS of dtJ/dtJ, vb/vb and wr/wr, almost all lipids were significantly decreased. The CNS of dtJ/dtJ and vb/vb were normal; wr/wr had minor reductions of certain glycolipids and phospholipids. Six mutants had relatively minor lipid abnormalities in their myelinated tissues. In cr/cr PNS, there were elevated levels of the cerebrosides and major phospholipids; the CNS was virtually normal. In db/db CNS and PNS, there were reduced levels of the nonhydroxy forms of cerebroside and sulfatide. The major change in htr/htr was the elevation of all the glycolipids in the CNS. In the CNS of Lc/+, nonhydroxy cerebroside was reduced. In shm/shm PNS, nonhydroxy sulfatide was elevated and there were small decreases in some of the phospholipids. wl/wl CNS showed decreases among most of the glycolipids. Mutants homozygous for du, mto, spa and tg had virtually normal lipid levels in both the optic and peripheral nerves. Cholesterol ester, lysophospholipids and other unusual lipid species were not detected in any of the mutants. The plasmalogen forms of ethanolamine and choline phosphatides were at normal levels in all mutants that otherwise had significant alterations among their lipids. Although many alterations in lipid composition were found in these mutants, the changes were moderate compared to the classical myelin mutants and indicate that none of the mutants are severely myelin-deficient.(ABSTRACT TRUNCATED AT 400 WORDS)
通过薄层色谱法分析了可能存在髓鞘异常的神经学突变小鼠的白质和周围神经的脂质,并通过光密度法进行定量。在检查的中枢神经系统(CNS)和/或周围神经系统(PNS)组织(视神经、三叉神经和坐骨神经)中,八个突变体存在主要异常。在axJ/axJ的视神经中,主要磷脂水平增加了20%-30%;周围神经正常。在bc3J/bc3J的中枢神经系统中,主要磷脂和胆固醇增加了25%-40%;周围神经正常。在myd/myd的中枢神经系统中,两种形式的脑苷脂和主要磷脂水平均增加了约20%;在周围神经系统中,脂质正常。ot/ot的中枢神经系统中,所有糖脂减少了20%-40%,一些磷脂和胆固醇有轻微改变;周围神经系统中,两种形式的脑苷脂减少了20%。在ji/ji的周围神经系统中,糖脂减少了10%-40%,三种主要磷脂减少了15%-25%;中枢神经系统基本正常。在dtJ/dtJ、vb/vb和wr/wr的周围神经系统中,几乎所有脂质都显著减少。dtJ/dtJ和vb/vb的中枢神经系统正常;wr/wr的某些糖脂和磷脂有轻微减少。六个突变体在其有髓组织中的脂质异常相对较小。在cr/cr的周围神经系统中,脑苷脂和主要磷脂水平升高;中枢神经系统基本正常。在db/db的中枢神经系统和周围神经系统中,脑苷脂和硫脂的非羟基形式水平降低。htr/htr的主要变化是中枢神经系统中所有糖脂升高。在Lc/+的中枢神经系统中,非羟基脑苷脂减少。在shm/shm的周围神经系统中,非羟基硫脂升高,一些磷脂有小幅减少。wl/wl的中枢神经系统中,大多数糖脂减少。du、mto、spa和tg的纯合突变体在视神经和周围神经中的脂质水平基本正常。在任何突变体中均未检测到胆固醇酯、溶血磷脂和其他异常脂质种类。在脂质有显著改变的所有突变体中,乙醇胺和胆碱磷脂的缩醛磷脂形式水平正常。尽管在这些突变体中发现了许多脂质组成的改变,但与经典的髓鞘突变体相比,这些变化较为温和,表明没有一个突变体严重缺乏髓鞘。(摘要截断于400字)