Molecular physiology and pathology of the nucleotide sugar transporter family (SLC35)
Molecular physiology and pathology of the nucleotide sugar transporter family (SLC35)
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DOI：
10.1007/s00424-003-1093-0
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发表时间：
2004-02-01
影响因子：
4.5
通讯作者：
Kawakita, M
中科院分区：
文献类型：
--
作者：
Ishida, N;Kawakita, M
溶质载体家族SLC 35由人体中至少17种分子种类组成。迄今为止表征的家族成员编码定位于高尔基体和/或内质网（ER）的核苷酸糖转运蛋白。这些转运蛋白将胞质中汇集的核苷酸糖转运到这些细胞器的内腔中，在那里发生大多数糖缀合物的合成。病理分析和发育研究的小，多细胞生物缺乏核苷酸糖转运蛋白已表明这些转运蛋白参与肿瘤转移，细胞免疫，器官发生和形态发生。白细胞粘附缺陷II型（LAD II）或糖基化IIc型（CDG IIc）先天性疾病是迄今已知的唯一由GDP-岩藻糖转运缺陷引起的人类先天性疾病。沿着LAD II，还讨论了核苷酸糖转运蛋白在结缔组织和肌肉疾病中的可能参与。
The solute carrier family SLC35 consists of at least 17 molecular species in humans. The family members so far characterized encode nucleotide sugar transporters localizing at the Golgi apparatus and/or the endoplasmic reticulum (ER). These transporters transport nucleotide sugars pooled in the cytosol into the lumen of these organelles, where most glycoconjugate synthesis occurs. Pathological analyses and developmental studies of small, multicellular organisms deficient in nucleotide sugar transporters have shown these transporters to be involved in tumour metastasis, cellular immunity, organogenesis and morphogenesis. Leukocyte adhesion deficiency type II (LAD II) or the congenital disorder of glycosylation type IIc (CDG IIc) are the sole human congenital disorders known to date that are caused by a defect of GDP-fucose transport. Along with LAD II, the possible involvement of nucleotide sugar transporters in disorders of connective tissues and muscles is also discussed.