P08237.2
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
93,254 Da
NCBI Official Full Name
ATP-dependent 6-phosphofructokinase, muscle type
NCBI Official Synonym Full Names
phosphofructokinase, muscle
NCBI Official Synonym Symbols
GSD7; PFK1; PFKA; PFKX; PFK-1; ATP-PFK; PPP1R122 [Similar Products]
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
UniProt Synonym Gene Names
UniProt Entry Name
PFKAM_HUMAN
NCBI Summary for PFKM
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 Comments for PFKM
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.
Protein type: EC 2.7.1.11; Carbohydrate Metabolism - fructose and mannose; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Kinase, other; Carbohydrate Metabolism - pentose phosphate pathway; Carbohydrate Metabolism - galactose
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; protein binding; protein C-terminus binding
Biological Process: fructose 6-phosphate metabolic process; glycolysis; muscle maintenance; protein oligomerization
Disease: Glycogen Storage Disease Vii
Research Articles on PFKM
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Pathways associated with PFKM blocking peptide
Diseases associated with PFKM blocking peptide
Organs/Tissues associated with PFKM blocking peptide
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