Anti-PPA2 Antibody Picoband®, Cy3 Conjugate

Boster Bio Anti-PPA2 Antibody Picoband® catalog # A06587. Tested in ELISA, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

Inorganic pyrophosphatase 2, mitochondrial is is a protein that in humans is encoded by the PPA2 gene. It is mapped to 4q24. The protein encoded by this gene is localized to the mitochondrion, is highly similar to members of the inorganic pyrophosphatase (PPase) family, and contains the signature sequence essential for the catalytic activity of PPase. PPases catalyze the hydrolysis of pyrophosphate to inorganic phosphate, which is important for the phosphate metabolism of cells. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Synonyms

Inorganic pyrophosphatase 2, mitochondrial; Pyrophosphatase SID6-306; Pyrophosphate phospho-hydrolase 2; PPase 2; PPA2; HSPC124

Gene Name

pyrophosphatase (inorganic) 2

Gene ID

27068

UniProt

Q9H2U2

Host

Rabbit

Reactivity

Human,Mouse

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E.coli-derived human PPA2 recombinant protein (Position: A33-K334).

Clonality

Polyclonal

Tissue Specificity

Detected in brain, gastric carcinoma, lung, ovary, skeletal muscle, umbilical cord blood and a cell line derived from kidney proximal tubule epithelium.

Applications

ELISA,IF,IHC,ICC,WB

Purification

Immunogen affinity purified.

Concentration

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

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Function

Hydrolyzes inorganic pyrophosphate. This activity is essential for correct regulation of mitochondrial membrane potential, and mitochondrial organization and function.

Components

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

References & Citations

1. Guimier, A., Gordon, C. T., Godard, F., Ravenscroft, G., Oufadem, M., Vasnier, C., Rambaud, C., Nitschke, P., Bole-Feysot, C., Masson, C., Dauger, S., Longman, C., and 15 others. Biallelic PPA2 mutations cause sudden unexpected cardiac arrest in infancy. Am. J. Hum. Genet. 99: 666-673, 2016. 2. Kennedy, H., Haack, T. B., Hartill, V., Matakovic, L., Baumgartner, E. R., Potter, H., Mackay, R., Alston, C. L., O'Sullivan, S., McFarland, R., Connolly, G., Gannon, C., and 23 others. Sudden cardiac death due to deficiency of the mitochondrial inorganic pyrophosphatase PPA2. Am. J. Hum. Genet. 99: 674-682, 2016.

Storage Conditions

Store 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 freeze-thaw cycles.