糖基磷脂酰肌醇锚定生物合成
中文名称
通路描述
细胞表面的蛋白可以通过称为糖基磷脂酰肌醇（GPI）锚定的糖脂结构连接到细胞膜上。在多种真核生物中，包括原生动物、真菌和哺乳动物，已鉴定出数百种GPI锚定蛋白。所有与蛋白结合的GPI锚定结构具有共同的核结构，其特征是Man (a1-4) GlcN (a1-6) myo-inositol-1P - 脂质。GPI锚定生物合成的过程分为三个阶段：(i) GPI前体在ER膜中的预组装，(ii) GPI与ER腔内新合成蛋白的C-末端结合，以及(iii) ER和Golgi中的脂质重塑和/或糖侧链修饰。GPI锚定生物合成基因缺陷会导致遗传性疾病，即阵发性夜间血红蛋白尿症。
英文描述
Cell surface proteins can be attached to the cell membrane via the glycolipid structure called glycosylphosphatidylinositol (GPI) anchor. Hundreds of GPI-anchored proteins have been identified in many eukaryotes ranging from protozoa and fungi to mammals. All protein-linked GPI anchors share a common core structure, characterized by the substructure Man (a1-4) GlcN (a1-6) myo-inositol-1P-lipid. Biosynthesis of GPI anchors proceeds in three stages: (i) preassembly of a GPI precursor in the ER membrane, (ii) attachment of the GPI to the C-terminus of a newly synthesized protein in the lumen of the ER, and (iii) lipid remodeling and/or carbohydrate side-chain modifications in the ER and the Golgi. Defects of GPI anchor biosynthesis gene result in a genetic disorder, paroxysmal nocturnal hemoglobinuria.
所含基因
30 个基因