Description
| 抗体名: | PFKM Antibody |
| 抗体コード: | PACO01322 |
| サイズ: | 50ug |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB, IHC |
| 推奨される希釈: | WB:1:500-1:2000, IHC:1:100-1:300 |
| 反応性: | Human, Mouse, Rat |
| 免疫原: | synthesized peptide derived from the Internal region of human PFKM. |
| 憲法: | Liquid |
| ストレージバッファ: | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| 精製方法: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
| シノニム: | PFKM; PFKX; 6-phosphofructokinase; muscle type; Phosphofructo-1-kinase isozyme A; PFK-A; Phosphofructokinase-M; Phosphofructokinase 1; Phosphohexokinase |
| UniProt Protein Function: | PFKM: phosphofructokinase, muscle type. An ubiquitous metabolic enzyme involved in the synthesis and degradation of fructose 2,6-bisphosphate. Key control step of glycolysis. An allosteric enzyme activated by ADP, AMP, or fructose bisphosphate and inhibited by ATP or citrate. Activity: ATP D-fructose 6-phosphate = ADP D-fructose 1,6-bisphosphate. The holoenzyme consists of 4 subunits. The liver and muscle enzymes are homo-tetramers of four liver or muscle isoforms, respectively. The red blood cell enzyme consists hetero-tetramers of the muscle and liver isoforms. A subunit composition with a higher proportion of platelet type subunits is found in platelets, brain and fibroblasts. Defects in PFKM are the cause of glycogen storage disease VII (GSD-VII) also known as Tarui disease. Two alternatively spliced isoforms have been described. |
| UniProt Protein Details: | Protein type:Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - pentose phosphate pathway; EC 2.7.1.11; Carbohydrate Metabolism - fructose and mannose; Kinase, other Chromosomal Location of Human Ortholog: 12q13.3 Cellular Component: 6-phosphofructokinase complex; apical plasma membrane; cytosol Molecular Function:6-phosphofructokinase activity; ATP binding; identical protein binding; kinase binding; metal ion binding; protein binding; protein C-terminus binding; protein homodimerization activity Biological Process: carbohydrate metabolic process; carbohydrate phosphorylation; fructose 6-phosphate metabolic process; glucose homeostasis; glucose metabolic process; glycogen catabolic process; glycolysis; muscle maintenance; positive regulation of insulin secretion; protein oligomerization Disease: Glycogen Storage Disease Vii |
| NCBI Summary: | 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] |
| UniProt Code: | P08237 |
| NCBI GenInfo Identifier: | 125126 |
| NCBI Gene ID: | 5213 |
| NCBI Accession: | P08237.2 |
| UniProt Secondary Accession: | P08237,Q16814, Q16815, Q6ZTT1, J3KNX3, |
| UniProt Related Accession: | P08237 |
| Molecular Weight: | 93,254 Da |
| NCBI Full Name: | ATP-dependent 6-phosphofructokinase, muscle type |
| NCBI Synonym Full Names: | phosphofructokinase, muscle |
| NCBI Official Symbol: | PFKM |
| NCBI Official Synonym Symbols: | GSD7; PFK1; PFKA; PFKX; PFK-1; ATP-PFK; PPP1R122 |
| NCBI Protein Information: | ATP-dependent 6-phosphofructokinase, muscle type |
| UniProt Protein Name: | ATP-dependent 6-phosphofructokinase, muscle type |
| UniProt Synonym Protein Names: | 6-phosphofructokinase type A; Phosphofructo-1-kinase isozyme A; PFK-A; Phosphohexokinase |
| Protein Family: | ATP-dependent 6-phosphofructokinase |
| UniProt Gene Name: | PFKM |
| UniProt Entry Name: | PFKAM_HUMAN |
