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Anti-PPA2 Antibody Picoband® Fluoro594 Conjugated

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

PPA2

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

Flow Cytometry

Field of Research

Cofactors, Vitamins/Minerals, Metabolism, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Pathways and Processes, Signal Transduction, Vitamins/Minerals

Purification

Immunogen affinity purified.

Form

Liquid

Function

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

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

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Product Datasheet

https://www.bosterbio.com/datasheet?sku=A06587-Fluoro594

Calculated Molecular Weight

37,920 MW

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Pyrophosphatase (inorganic) 2

Subcellular Location

Mitochondrion.

Protein Name

AP-2 complex subunit beta

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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