半乳糖代谢
中文名称
通路描述
Leloir 半乳糖代谢途径将膳食半乳糖(来自乳糖)转化为 UDP-葡萄糖,作为能量来源或生物合成前体。四个酶——半乳糖激酶、半乳糖 -1-磷酸尿苷酰转移酶和 UDP-半乳糖异构酶——至关重要;缺陷会导致半乳糖血症,伴有严重的发育后果。
英文描述
The Leloir pathway of galactose metabolism converts dietary galactose (from lactose in milk) into UDP-glucose for use as an energy source or biosynthetic precursor. The four enzymes — galactokinase, galactose-1-phosphate uridylyltransferase, and UDP-galactose epimerase — are essential; defects cause galactosemia with severe developmental consequences.
所含基因
32 个基因