antibody

PFKM antibody (clone AT2F11)

MBS200252

0.05 mL

240 USD

monoclonal

mouse

nonconjugated

AT2F11

western blot, ELISA, enzyme immunoassay

Antibody

PFKM antibody (clone AT2F11)

PFKM

GSD7; PFK-1; PFK1; PFKA; PFKX; 6-Phosphofructokinase; muscle type 6 Phosphofructokinase Muscle Type; EC 2.7.1.11; MGC8699; PFKL; PFKM; PFKP; Phosphofructo 1 Kinase Isozyme A; Phosphofructokinase 1; Phosphofructokinase M; Phosphofructokinase; muscle; Phosphofructokinase; muscle type; Phosphofructokinase; polypeptide X; Phosphohexokinase.

ATP-dependent 6-phosphofructokinase, muscle type isoform 2; ATP-dependent 6-phosphofructokinase, muscle type; ATP-dependent 6-phosphofructokinase, muscle type; 6-phosphofructo-1-kinase; 6-phosphofructokinase type A; ATP-PFK; PFK-A; phosphofructo-1-kinase isozyme A; phosphofructokinase 1; phosphofructokinase, polypeptide X; phosphofructokinase-M; phosphohexokinase; protein phosphatase 1, regulatory subunit 122; phosphofructokinase, muscle; 6-phosphofructokinase type APhosphofructo-1-kinase isozyme A

PFKM

PFKM; PFKM; GSD7; PFK1; PFKA; PFKX; PFK-1; ATP-PFK; PPP1R122; ; PFK-M

PFKAM_HUMAN

Monoclonal

IgG2a,k

AT2F11

Mouse

Human

780

By protein-A affinity chromatography

Liquid. Supplied in Phosphate-Buffered Saline (pH 7.4) containing 10% Glycerol, 0.01% Sodium Azide.

1mg/ml

Can be stored at 4 degree C. For long term storage, aliquot and store at -20 degree C. Avoid repeated freezing and thawing cycles.

ELISA (EIA), Western Blot (WB)

The antibody has been tested by ELISA, Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results. Recommended dilution range for Western blot analysis is 1:500 ~ 1:2000. Recommended starting dilution is 1:2000.

Antigen Species: Human. Clone: Anti-human PFKM mAb, clone AT2F11, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human PFKM protein. Immunogen: Recombinant human PFKM (1-780aa) purified from E Coli

Metabolism

PFKM(phosphofructokinase, muscle) is a regulatory glycolytic enzyme that convert fructose 6-phosphate and ATP into fructose 1,6-bisphosphate (through PFK-1), fructose 2,6-bisphosphate (through PFK-2) and ADP. Three phosphofructokinase isozymes exist in humans: muscle, liver and platelet. Mutations in this gene have been associated with glycogen storage disease type VII, also known as Tarui disease.

Martinez-Costa OH., et al. (2007) FEBS Lett. 581(16):3033-8. Kim JW., et al. (2004) Mol Cell Biol. 24(13):5923-36

4505749

NP_000280

NM_000289.5

93,254 Da

AMPK Signaling Pathway (989139); AMPK Signaling Pathway (992181); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Central Carbon Metabolism In Cancer Pathway (1059538); Central Carbon Metabolism In Cancer Pathway (1084231); Disease Pathway (530764); Fructose And Mannose Metabolism Pathway (82930)

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]

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 - pentose phosphate pathway; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - fructose and mannose; Carbohydrate Metabolism - galactose; Kinase, other. Chromosomal Location of Human Ortholog: 12q13.3. Cellular Component: 6-phosphofructokinase complex; apical plasma membrane; cytosol. Molecular Function: protein C-terminus binding; identical protein binding; protein binding; protein homodimerization activity; metal ion binding; kinase binding; ATP binding; 6-phosphofructokinase activity. Biological Process: muscle maintenance; glycolysis; carbohydrate metabolic process; carbohydrate phosphorylation; positive regulation of insulin secretion; glucose metabolic process; pathogenesis; glucose homeostasis; fructose 6-phosphate metabolic process; protein oligomerization. Disease: Glycogen Storage Disease Vii

0.05 mL

240 USD

0.1 mL

315