半岛bd体育手机客户端 说明
一般描述
Phosphoglucomutase-1 (PGM1) is an evolutionarily conserved enzyme that belongs to the phosphohexose mutase family. It is the major isoform of PGM in skeletal muscle and most other tissues. The PGM1 gene is mapped to human chromosome 1p31.3.
生化/生理作用
Phosphoglucomutase-1 (PGM1) catalyzes the bidirectional interconversion of glucose-1-phosphate (G-1-P) and glucose-6-phosphate (G-6-P). It regulates carbohydrate metabolism, energy production, and protein N-glycosylation. PGM1 participates in the biosynthesis of nucleotide sugars required for glycan biosynthesis. Variation in the PGM1 gene leads to PGM1 deficiency, which is considered an inherited metabolic disorder in humans. PGM1 deficiency causes autosomal recessive diseases such as glycogen storage disease type XIV and congenital disorder of protein N-glycosylation. Affected patients show multiple disease phenotypes, reflecting the central role of the enzyme in glucose homeostasis. The influence of PGM1 deficiency on protein glycosylation patterns is also widespread. PGM1 acts as a metabolic tumor suppressor.
单位定义
One unit will convert 1.0 μmole of α-
外形
Supplied as a lyophilized powder containing tris-buffered saline, EDTA, DTT and stabilizer.
基本信息
| 酶学委员会(EC)编号 | 5.4.2.2 ( BRENDA | IUBMB ) |
| NACRES | NA.54 |
半岛bd体育手机客户端 性质
| 质量水平 | 200 |
| 重组 | expressed inE. coli |
| 描述 | PGM1 isoform sequence with a predicted molecular mass of 61.3kDa. |
| 测定 | ≥95% (PAGE) |
| 形式 | lyophilized powder |
| specific activity | ≥400 units/mg protein |
| 运输 | dry ice |
| 储存温度 | ?20℃ |
安全信息
| 储存分类代码 | 11 - Combustible Solids |
| WGK | WGK 2 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
Sigma-Aldrich
m.cnreagent.com
