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人类大脑发育受损:先天性甲状腺功能减退症中的糖缀合物和脂质变化

Impairment of human brain development: glycoconjugate and lipid changes in congenital athyroidism.

作者信息

Annunziata P, Federico A, D'Amore I, Corona R M, Guazzi G C

出版信息

Early Hum Dev. 1983 Oct;8(3-4):269-78. doi: 10.1016/0378-3782(83)90009-9.

DOI:10.1016/0378-3782(83)90009-9
PMID:6227470
Abstract

The brain glycoconjugates, glycosidases and lipids have been studied in a case of human congenital athyroidism never treated with hormonal replacement. Increase of membrane-bound mucopolysaccharides has been found in the grey matter. Total lipids in the white matter, and phospholipids, and total glycolipids and cholesterol in both white and grey matter were reduced. Sialic acid (NeuNAc) and hexosamines in the cytosol grey matter were significantly raised, while they were reduced in the membrane-bound grey fraction. These biochemical findings confirm impairment of the myelination process in human hypothyroidism as is well established in experimental hypothyroidism. The significant changes in the carbohydrate moieties of the brain glycoproteins focus attention on complex alterations of structure and function of the cell plasma membranes.

摘要

对一例从未接受过激素替代治疗的人类先天性甲状腺功能减退症患者的脑糖缀合物、糖苷酶和脂质进行了研究。在灰质中发现膜结合粘多糖增加。白质中的总脂质、磷脂以及白质和灰质中的总糖脂和胆固醇均减少。细胞溶质灰质中的唾液酸(NeuNAc)和己糖胺显著升高,而膜结合灰质部分中的则减少。这些生化发现证实了人类甲状腺功能减退症中髓鞘形成过程受损,这在实验性甲状腺功能减退症中已得到充分证实。脑糖蛋白碳水化合物部分的显著变化使人们关注细胞质膜结构和功能的复杂改变。

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