Anti-PFKM Antibody Picoband®

SKU: A00437-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: ELISA,IF,IHC,ICC,WB,Flow Cytometry

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: PFKM

Specificity:

Background: 6-phosphofructokinase, muscle type is an enzyme that in humans is encoded by the PFKM gene on chromosome 12. 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.

Immunogen: E.coli-derived human PFKM recombinant protein (Position: R39-V780). Human PFKM shares 97.7% and 96.8% amino acid (aa) sequence identity with mouse and rat PFKM, respectively.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Ashley, P. L., Price, E. R., Lebo, R. V., Cox, D. R. An expressed phosphofructokinase gene (PFKX) maps to human chromosome 12 and mouse chromosome 15. (Abstract) Cytogenet. Cell Genet. 46: 573 only, 1987. 2. Bruno, C., Minetti, C., Shanske, S., Morreale, G., Bado, M., Cordone, G., DiMauro, S. Combined defects of muscle phosphofructokinase and AMP deaminase in a child with myoglobinuria. Neurology 50: 296-298, 1998. 3. Hamaguchi, T., Nakajima, H., Noguchi, T., Nakagawa, C., Kuwajima, M., Kono, N., Tarui, S., Matsuzawa, Y. Novel missense mutation (W686C) of the phosphofructokinase-M gene in a Japanese patient with a mild form of glycogenosis VII. Hum. Mutat. 8: 273-275, 1996.

Uniprot ID: P08237

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross reactivity with other proteins.

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 85 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 5213

Protein Name: ATP-dependent 6-phosphofructokinase, muscle type

Gene Full Name: phosphofructokinase, muscle

Synonyms: GSD7; PFK 1; PFK A; PFK M; PFKM; PFKX; Phosphofructokinase 1; Phosphofructokinase M; phosphofructokinase; muscle; Phosphohexokinase