Anti-PFKM Antibody (CAB5477)
- SKU:
- CAB5477
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
Antibody Name: | Anti-PFKM Antibody |
Antibody SKU: | CAB5477 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC IF |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 501-780 of human PFKM (NP_001160160.1). |
Application: | WB IHC IF |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | 22Rv1, HeLa, SW620, K-562, 293T, Mouse heart, Mouse brain |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 501-780 of human PFKM (NP_001160160.1). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | AYTG GLEL MEGR KQFD ELCI PFVV IPAT VSNN VPGS DFSV GADT ALNT ICTT CDRI KQSA AGTK RRVF IIET MGGY CGYL ATMA GLAA GADA AYIF EEPF TIRD LQAN VEHL VQKM KTTV KRGL VLRN EKCN ENYT TDFI FNLY SEEG KGIF DSRK NVLG HMQQ GGSP TPFD RNFA TKMG AKAM NWMS GKIK ESYR NGRI FANT PDSG CVLG MRKR ALVF QPVA ELKD QTDF EHRI PKEQ WWLK LRPI LKIL AKYE IDLD TSDH AHLE HITR KRSG EAAV |
Gene ID: | 5213 |
Uniprot: | P08237 |
Cellular Location: | Cytoplasm |
Calculated MW: | 81kDa/85kDa/93kDa |
Observed MW: | 85kDa |
Synonyms: | PFKM, ATP-PFK, GSD7, PFK-1, PFK1, PFKA, PFKX, PPP1R122 |
Background: | 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. |
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 |