人 Fructose 6 Phosphate Kinase 抗体对
01AB01788
Phosphofructokinase, muscle type (PFKM), is an 85 kDa enzyme subunit encoded by the PFKM gene on chromosome 12 and is a key component of the tetrameric phosphofructokinase complex that catalyzes the phosphorylation of fructose 6-phosphate to fructose 1,6-bisphosphate, the major rate-limiting and committed step of glycolysis in muscle and other tissues. The PFKM subunit is predominantly expressed in skeletal muscle, but also contributes to enzyme complexes in erythrocytes, heart, brain, and testis, with tissue-specific tetramer composition. Structurally, the N-terminal region of PFKM is responsible for catalytic activity, while the C-terminal contains allosteric ligand binding sites. Mutations in PFKM cause glycogen storage disease type VII (Tarui disease). The severity of symptoms can vary, and affected individuals may experience muscle breakdown (rhabdomyolysis) after moderate exertion. Overexpression of PFKM has also been linked to metabolic disturbances such as insulin resistance in type 2 diabetes.
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技术参数
- 靶点基因名
- PFKM
- 基因别名
- PFKX
- 蛋白别名
- phosphofructokinase, muscle; Fructose 6 Phosphate Kinase
- UniProt
- P08237
- 物种
- Human
产品详情
- 靶点名称
- Fructose 6 Phosphate Kinase
- 蛋白名称
- phosphofructokinase, muscle
- 蛋白别名
- ATP-dependent 6-phosphofructokinase; muscle type; ATP-PFK; PFK-M; 6-phosphofructokinase type A; Phosphofructo-1-kinase isozyme A; PFK-A; Phosphohexokinase
- 靶点基因名
- PFKM
- 基因别名
- PFKX
- UniProt
- P08237
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Phosphofructokinase, muscle type (PFKM), is an 85 kDa enzyme subunit encoded by the PFKM gene on chromosome 12 and is a key component of the tetrameric phosphofructokinase complex that catalyzes the phosphorylation of fructose 6-phosphate to fructose 1,6-bisphosphate, the major rate-limiting and committed step of glycolysis in muscle and other tissues. The PFKM subunit is predominantly expressed in skeletal muscle, but also contributes to enzyme complexes in erythrocytes, heart, brain, and testis, with tissue-specific tetramer composition. Structurally, the N-terminal region of PFKM is responsible for catalytic activity, while the C-terminal contains allosteric ligand binding sites. Mutations in PFKM cause glycogen storage disease type VII (Tarui disease). The severity of symptoms can vary, and affected individuals may experience muscle breakdown (rhabdomyolysis) after moderate exertion. Overexpression of PFKM has also been linked to metabolic disturbances such as insulin resistance in type 2 diabetes.