SAE0022

Phosphoglucomutase 1 human

lyophilized powder, recombinant, expressed in E. coli

• EC Number: 5.4.2.2 (BRENDA, IUBMB)
• UNSPSC Code: 12352204
• NACRES: NA.54

Properties

• recombinant: expressed in E. coli
• Quality Level: 200
• description: PGM1 isoform sequence with a predicted molecular mass of 61.3kDa.
• Assay: ≥95% (PAGE)
• form: lyophilized powder
• specific activity: ≥400 units/mg protein
• shipped in: dry ice
• storage temp.: −20°C

Description

General description

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.

Biochem/physiol Actions

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.

Unit Definition

One unit will convert 1.0 μmole of α-D-Glucose-1-Phosphate to α-D-Glucose-6-phosphate per minute at pH 7.4 at 30 °C.

Physical form

Supplied as a lyophilized powder containing tris-buffered saline, EDTA, DTT and stabilizer.

Safety Information

• Storage Class Code: 11 - Combustible Solids
• WGK: WGK 2
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable