Huether G, Klapproth M, Neuhoff V
Neurochem Res. 1986 Sep;11(9):1303-11. doi: 10.1007/BF00966124.
The fatty acid composition of individual myelin lipids from rat forebrain and spinal cord was analysed at 20 and 30 days p.p. During this phase of rapid myelination the proportions of C 16:0 and C 18:0 fatty acids decreased whereas the relative amounts of long chain and unsaturated fatty acids increased in most lipid classes. This developmental shift was more pronounced in the forebrain and was different with respect to both magnitude and direction for each myelin lipid. The experimental induction of chronic hyperphenylalaninaemia (hyper-Phe) from day 3 p.p. lead to alterations in the rate of myelination, which were most pronounced in the forebrain. At 20 days, especially in the forebrain, chain elongation and desaturation of fatty acids were delayed. This delay was maximal in sphingomyelin fatty acids, C 18:0 and C 24:1. In hydroxycerebrosides, the shift in the fatty acid composition from C 16:0 to C 22:0 was accelerated in hyper-Phe rats. No significant difference in the fatty acid composition of any myelin lipid was found in the more mature myelin (spinal cord, 30 days p.p.). No evidence was found of a primary effect of hyper-Phe on myelination.
在出生后20天和30天时，对大鼠前脑和脊髓中单个髓磷脂脂质的脂肪酸组成进行了分析。在这个快速髓鞘形成阶段，大多数脂质类别中C16:0和C18:0脂肪酸的比例下降，而长链和不饱和脂肪酸的相对含量增加。这种发育变化在前脑中更为明显，并且每种髓磷脂脂质在幅度和方向上都有所不同。从出生后第3天开始实验诱导慢性高苯丙氨酸血症（高苯丙氨酸）会导致髓鞘形成速率改变，这在前脑中最为明显。在20天时，尤其是在前脑中，脂肪酸的链延长和去饱和被延迟。这种延迟在鞘磷脂脂肪酸、C18:0和C24:1中最为显著。在羟基脑苷脂中，高苯丙氨酸大鼠中脂肪酸组成从C16:0到C22:0的转变加速。在更成熟的髓鞘（脊髓，出生后30天）中，未发现任何髓磷脂脂质的脂肪酸组成有显著差异。未发现高苯丙氨酸对髓鞘形成有主要影响的证据。
