Human PFKM Recombinant Protein (RPPB2600)
- SKU:
- RPPB2600
- Product type:
- Recombinant Protein
- Size:
- 20ug
- Species:
- Human
- Target:
- PFKM
- Synonyms:
- EC 27111
- GSD7
- PFK-1
- PFK1
- Source:
- Escherichia Coli
- Uniprot:
- P08237
Description
| Product Name: | Human PFKM Recombinant Protein |
| Product Code: | RPPB2600 |
| Size: | 20µg |
| Species: | Human |
| Target: | PFKM |
| Synonyms: | EC 2.7.1.11, GSD7, PFK-1, PFK1, PFKA, PFKX, Phosphofructokinase-M, Phosphofructokinase 1, Phosphohexokinase, Phosphofructo-1-kinase isozyme A, MGC8699, PFKM. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile filtered colorless solution. |
| Formulation: | PFKM Human solution containing 20mM Trsi HCl pH-8, 5mM DTT, 0.2M NaCl and 20% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Please prevent freeze thaw cycles. |
| Purity: | Greater than 80% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MTHEEHHAAK TLGIGKAIAV LTSGGDAQGM NAAVRAVVRV GIFTGARVFF VHEGYQGLVD GGDHIKEATW ESVSMMLQLG GTVIGSARCK DFREREGRLR AAYNLVKRGI TNLCVIGGDG SLTGADTFRS EWSDLLSDLQ KAGKITDEEA TKSSYLNIVG LVGSIDNDFC GTDMTIGTDS ALHRIMEIVD AITTTAQSHQ RTFVLEVMGR HCGYLALVTS LSCGADWVFI PECPPDDDWE EHLCRRLSET RTRGSRLNII IVAEGAIDKN GKPITSEDIK NLVVKRLGYD TRVTVLGHVQ RGGTPSAFDR ILGSRMGVEA VMALLEGTPD TPACVVSLSG NQAVRLPLME CVQVTKDVTK AMDEKKFDEA LKLRGRSFMN NWEVYKLLAH VRPPVSKSGS HTVAVMNVGA PAAGMNAAVR STVRIGLIQG NRVLVVHDGF EGLAKGQIEE AGWSYVGGWT GQGGSKLGTK RTLPKKSFEQ ISANITKFNI QGLVIIGGFE AYTGGLELME GRKQFDELCI PFVVIPATVS NNVPGSDFSV GADTALNTIC TTCDRIKQSA AGTKRRVFII ETMGGYCGYL ATMAGLAAGA DAAYIFEEPF TIRDLQANVE HLVQKMKTTV KRGLVLRNEK CNENYTTDFI FNLYSEEGKG IFDSRKNVLG HMQQGGSPTP FDRNFATKMG AKAMNWMSGK IKESYRNGRI FANTPDSGCV LGMRKRALVF QPVAELKDQT DFEHRIPKEQ WWLKLRPILK ILAKYEIDLD TSDHAHLEHI TRKRSGEAAV |
PFKM is a regulatory glycolytic enzyme that converts 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 PFKM gene have been related with glycogen storage disease type VII, also identified as Tarui disease.
PFKM Human Recombinant produced in E.Coli is a single, non-glycosylated,polypeptide chain containing 800 amino acids (1-780 a.a.) and having a molecular mass of 87.3 kDa. PFKM protein is fused to a 20 amino acid His-Tag at N-terminus and purified by standard chromatography.
| 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 |
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