FPGT - fucose-1-phosphate guanylyltransferase Gene

中文名称：1-磷酸岩藻糖鸟苷酸转移酶

种属: Homo sapiens

同用名: GFPP

关于 FPGT

功能概要

L-岩藻糖是糖蛋白和其他复杂碳水化合物中的关键糖，因为它可能参与这些大分子的许多功能作用，例如细胞间识别。这些岩藻糖基化寡糖的岩藻糖基供体是 GDP-β-L-岩藻糖。 GDP-岩藻糖的生物合成有两种替代途径；主要途径将 GDP-α-D-甘露糖转化为 GDP-β-L-岩藻糖。由该基因编码的蛋白质参与了某些哺乳动物组织 (如肝脏和肾脏) 中存在的替代途径，并且似乎起到补救途径的作用，以重新利用糖蛋白和糖脂周转产生的 L-岩藻糖。该途径涉及 L-岩藻糖磷酸化形成 β-L-岩藻糖-1-磷酸，然后通过岩藻糖-1-磷酸鸟苷转移酶将 β-L-岩藻糖-1-磷酸与 GTP 缩合形成 GDP-β- L-岩藻糖。可变剪接导致多个转录本变体。该基因与邻近的下游 TNNI3 相互作用激酶 (TNNI3K) 基因之间也存在通读转录。[RefSeq 提供，2010 年 12 月]

L-fucose is a key sugar in glycoproteins and other complex Carbohydrates since it may be involved in many of the functional roles of these macromolecules, such as in cell-cell recognition. The fucosyl donor for these fucosylated oligosaccharides is GDP-beta-L-fucose. There are two alternate pathways for the biosynthesis of GDP-fucose; the major pathway converts GDP-alpha-D-mannose to GDP-beta-L-fucose. The protein encoded by this gene participates in an alternate pathway that is present in certain mammalian tissues, such as liver and kidney, and appears to function as a salvage pathway to reutilize L-fucose arising from the turnover of glycoproteins and glycolipids. This pathway involves the phosphorylation of L-fucose to form beta-L-fucose-1-phosphate, and then condensation of the beta-L-fucose-1-phosphate with GTP by fucose-1-phosphate guanylyltransferase to form GDP-beta-L-fucose. Alternative splicing results in multiple transcript variants. Read-through transcription also exists between this gene and the neighboring downstream TNNI3 interacting kinase (TNNI3K) gene. [provided by RefSeq, Dec 2010]