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Product Name PGAM2 (N-terminal) Chinese Name PGAM2(N端)抗体 Alias BPG dependent PGAM 2; BPG-dependent PGAM 2; GSD10; MGC88743; Muscle specific phosphoglycerate mutase; Muscle-specific phosphoglycerate mutase; OTTHUMP00000207787; PGAM 2; PGAM M; PGAM-M; Pgam2; PGAM2_HUMAN; PGAMM. Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) Applications WB=1:500-2000 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 29kDa Cellular localization The nucleus cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human PGAM2: 1-100/253 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Phosphoglycerate mutase (PGAM) catalyzes the reversible reaction of 3-phosphoglycerate (3-PGA) to 2-phosphoglycerate (2-PGA) in the glycolytic pathway. The PGAM is a dimeric enzyme containing, in different tissues, different proportions of a slow-migrating muscle (MM) isozyme, a fast-migrating brain (BB) isozyme, and a hybrid form (MB). This gene encodes muscle-specific PGAM subunit. Mutations in this gene cause muscle phosphoglycerate mutase eficiency, also known as glycogen storage disease X. [provided by RefSeq, Sep 2009]
Function:
Interconversion of 3- and 2-phosphoglycerate with 2,3-bisphosphoglycerate as the primer of the reaction. Can also catalyze the reaction of EC 5.4.2.4 (synthase) and EC 3.1.3.13 (phosphatase), but with a reduced activity.
Tissue Specificity:
In mammalian tissues there are two types of phosphoglycerate mutase isozymes: type-M in muscles and type-B in other tissues.
DISEASE:
Defects in PGAM2 are the cause of glycogen storage disease type 10 (GSD10) [MIM:261670]. A metabolic disorder characterized by myoglobinuria, increased serum creatine kinase levels, decreased phosphoglycerate mutase activity, myalgia, muscle pain, muscle cramps and excercise intolerance.
Similarity:
Belongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily.
SWISS:
P15259
Gene ID:
5224
Database links:
Entrez Gene: 5224 Human
Entrez Gene: 56012 Mouse
Omim: 612931 Human
SwissProt: P15259 Human
SwissProt: O70250 Mouse
Unigene: 23217 Cow
Unigene: 632642 Human
Unigene: 219627 Mouse
Unigene: 9738 Rat
Product Picture Sample:
Testis (Mouse) Lysate at 40 ug
Primary: Anti-PGAM2 (SL23501R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 29 kD
Observed band size: 29 kD
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