SAE0022
Phosphoglucomutase 1 human
lyophilized powder, recombinant, expressed in E. coli
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1 MG
$395.00
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$395.00
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recombinant
expressed in E. coli
Quality Level
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
General description
Biochem/physiol Actions
Phosphoglucomutase-1 (PGM1) catalyzes the bidirectional interconversion of glucose-1-phosphate (G-1-P) and glucose-6-phosphate (G-6-P).[1] It regulates carbohydrate metabolism, energy production, and protein N-glycosylation.[2] PGM1 participates in the biosynthesis of nucleotide sugars required for glycan biosynthesis.[5] Variation in the PGM1 gene leads to PGM1 deficiency, which is considered an inherited metabolic disorder in humans.[2][5] PGM1 deficiency causes autosomal recessive diseases such as glycogen storage disease type XIV and congenital disorder of protein N-glycosylation.[5] 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.[1][6] PGM1 acts as a metabolic tumor suppressor.[4]
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.
Storage Class
11 - Combustible Solids
wgk_germany
WGK 2
flash_point_f
Not applicable
flash_point_c
Not applicable
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Guang-Zhi Jin et al.
PLoS biology, 16(10), e2006483-e2006483 (2018-10-20)
Glycogen metabolism commonly altered in cancer is just beginning to be understood. Phosphoglucomutase 1 (PGM1), the first enzyme in glycogenesis that catalyzes the reversible conversion between glucose 1-phosphate (G-1-P) and glucose 6-phosphate (G-6-P), participates in both the breakdown and synthesis
Amichai Gutgold et al.
The Journal of clinical endocrinology and metabolism, 102(2), 345-349 (2016-10-19)
Hypoglycemia is a rare event in healthy adults, and the differential diagnosis includes many diseases, some of which are rare and easily missed. A 20-year-old male military paramedic was referred to our emergency department for investigation of recurrent hypoglycemia episodes
Wo-Tu Tian et al.
Neuromuscular disorders : NMD, 29(4), 282-289 (2019-02-10)
The congenital disorders of glycosylation are a group of clinically and biochemically heterogeneous diseases characterized by multisystem involvement due to glycosylation defect of protein and lipid. Here we report a 49-year-old man with exercise-induced fatigue and pain of muscle, tachypnea
Federica Conte et al.
Molecular genetics and metabolism, 131(1-2), 135-146 (2020-12-22)
Phosphoglucomutase 1 deficiency is a congenital disorder of glycosylation (CDG) with multiorgan involvement affecting carbohydrate metabolism, N-glycosylation and energy production. The metabolic management consists of dietary D-galactose supplementation that ameliorates hypoglycemia, hepatic dysfunction, endocrine anomalies and growth delay. Previous studies
Paul Hoff Backe et al.
Scientific reports, 10(1), 5656-5656 (2020-03-30)
Human phosphoglucomutase 1 (PGM1) is an evolutionary conserved enzyme that belongs to the ubiquitous and ancient α-D-phosphohexomutases, a large enzyme superfamily with members in all three domains of life. PGM1 catalyzes the bi-directional interconversion between α-D-glucose 1-phosphate (G1P) and α-D-glucose
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