ITT3680
ITT3680
- Catalog: ITT3680
- Gene/Protein: PFKM
- Product Description: Immunotag™ PFKM Polyclonal Antibody
385.0000
Price in reward points: 385
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Antibody Specification | |
Datasheet | |
Target Protein | PFKM |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,IHC-p,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/10000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Mouse,Rat |
Host Species | Rabbit |
Immunogen | Synthesized peptide derived from the Internal region of human PFKM |
Specificity | PFKM Polyclonal Antibody detects endogenous levels of PFKM protein. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | PFKM |
Accession No. | P08237 P47857 P47858 |
Alternate Names | PFKM; PFKX; 6-phosphofructokinase; muscle type; Phosphofructo-1-kinase isozyme A; PFK-A; Phosphofructokinase-M; Phosphofructokinase 1; Phosphohexokinase |
Description | phosphofructokinase, muscle(PFKM) Homo sapiens 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], |
Cell Pathway/ Category | Glycolysis / Gluconeogenesis,Pentose phosphate pathway,Fructose and mannose metabolism,Galactose metabolism, |
Protein Expression | Brain,Liver,Muscle,Skeletal muscle,Thymus, |
Subcellular Localization | cytoplasm,cytosol,6-phosphofructokinase complex,apical plasma membrane,extracellular exosome,sperm principal piece, |
Protein 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.,pathway:Carbohydrate degradation; glycolysis; D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 3/4.,similarity:Belongs to the phosphofructokinase family. Two domains subfamily.,subunit:Tetramer. Muscle is M4, liver is L4, and red cell is M3L, M2L2, or ML3., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |