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.
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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