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Schilder氏肾上腺脑白质营养不良是一种X-连锁的隐性遗传疾患,其特点是进行性神智障碍及肾上腺功能不全。本病的基本缺陷不明,但有长链饱和脂酸(23:0～30:0)贮积在脑与肾上腺的胆固醇酯、神经节苷脂及脑苷脂上。在培育的皮肤成纤维
Schilder’s Adrenoleukodystrophy is an X-linked recessive genetic disorder characterized by progressive mental retardation and adrenal insufficiency. The basic flaw is unknown, but long chain saturated fatty acids (23: 0-30: 0) store in the brain and adrenal cholesterol esters, gangliosides and cerebrosides. Fibers are cultivated on the skin