Target：PFKM

Fields：Glycolysis / Gluconeogenesis;Pentose phosphate pathway;Fructose and mannose metabolism;Galactose metabolism;Metabolic pathways;Carbon metabolism;Biosynthesis of amino acids;RNA degradation;HIF-1 signaling pathway;AMPK signaling pathway;Thyroid hormone signaling pathway;Glucagon signaling pathway;Central carbon metabolism in cancer

Gene Name：PFKM

Protein Name：6-phosphofructokinase muscle type

Human Gene Id：5213

Human Swiss Prot No：P08237

Mouse Gene Id：18642

Mouse Swiss Prot No：P47857

Rat Gene Id：65152

Rat Swiss Prot No：P47858

Immunogen：The antiserum was produced against synthesized peptide derived from human PFK-1. AA range:320-369

Specificity：PFKM Polyclonal Antibody detects endogenous levels of PFKM protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:10000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：PFKM;PFKX;6-phosphofructokinase; muscle type;Phosphofructo-1-kinase isozyme A;PFK-A;Phosphofructokinase-M;Phosphofructokinase 1;Phosphohexokinase

Observed Band(KD)：85kD

Background： Three phosphofructokinase isozymes exist in humans: muscle, liver and platelet. These isozymes function as subunits of the mammalian tetramer phosphofructokinase, which catalyzes the phosphorylation of fructose-6-phosphate to fructose-1,6-bisphosphate. Tetramer composition varies depending on tissue type. This gene encodes the muscle-type isozyme. Mutations in this gene have been associated with glycogen storage disease type VII, also known as Tarui disease. Alternatively spliced transcript variants have been described.[provided by RefSeq, Nov 2009],

Function：catalytic activity:ATP + D-fructose 6-phosphate = ADP + D-fructose 1,6-bisphosphate.,cofactor:Magnesium.,disease:Defects in PFKM are the cause of glycogen storage disease type 7 (GSD7) [MIM:232800]; also known as Tarui disease. GSD7 is an autosomal recessive disorder characterized by exercise intolerance with associated nausea and vomiting. Short bursts of intense activity are particularly difficult. Severe muscle cramps and myoglobinuria develop after vigorous exercise. Most patients obtain a "second wind" when the onset of exercise is followed by a brief rest period. In time patients adjust their activity level and are well compensated.,enzyme regulation:Allosteric enzyme activated by ADP, AMP, or fructose bisphosphate and inhibited by ATP or citrate.,miscellaneous:In human PFK exists as a system of 3 types of subunits, PFKM (muscle), PFKL (liver) and PFKP (platelet) isoenzymes.,pathwa

Subcellular Location：Cytoplasm .

Expression： Brain,Liver,Muscle,Skeletal muscle,Thymus,