Typical Testing Data/Standard Curve (for reference only)
Human 6-phosphofructokinase, muscle type ELISA Kit | PFKM elisa kit
Human 6-phosphofructokinase, muscle type ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
NCBI and Uniprot Product Information
NCBI Description
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 Description
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: Carbohydrate Metabolism - fructose and mannose; Kinase, other; Carbohydrate Metabolism - pentose phosphate pathway; EC 2.7.1.11; Carbohydrate Metabolism - glycolysis and gluconeogenesis; 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; 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
Research Articles on PFKM
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Product Notes
The Human PFKM pfkm (Catalog #AAA2886525) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2886525 ELISA Kit recognizes Human PFKM. It is sometimes possible for the material contained within the vial of "6-phosphofructokinase, muscle type, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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