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Mouse Anti-PPA2 Recombinant Antibody (7C1) (CBMAB-P2465-YC)

Provided herein is a Mouse monoclonal antibody against Human Pyrophosphatase (Inorganic) 2. The antibody can be used for immunoassay techniques, such as WB, IHC.
See all PPA2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
7C1
Antibody Isotype
IgG1
Application
WB, IHC

Basic Information

Immunogen
Full length human recombinant protein of human PPA2(NP_008834) produced in E. coli
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
PBS, pH 7.3, 1% BSA, 50% glycerol, 0.02% sodium azide
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Pyrophosphatase (Inorganic) 2
Introduction
PPA2 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.
Entrez Gene ID
27068
UniProt ID
Q9H2U2
Alternative Names
HSPC124; SID6-306
Function
Hydrolyzes inorganic pyrophosphate (PubMed:27523597).
This activity is essential for correct regulation of mitochondrial membrane potential, and mitochondrial organization and function (PubMed:27523598).
Biological Process
Diphosphate metabolic processManual Assertion Based On ExperimentIMP:UniProtKB
Phosphate-containing compound metabolic processManual Assertion Based On ExperimentIBA:GO_Central
Regulation of mitochondrial membrane potentialManual Assertion Based On ExperimentIMP:UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
Sudden cardiac failure, alcohol-induced (SCFAI):
An autosomal recessive disease characterized by sudden death due to unexpected cardiac arrest following ingestion of small amounts of alcohol.
Sudden cardiac failure, infantile (SCFI):
A disease characterized by sudden death within the first 2 years of life due to unexpected cardiac arrest. Some patients manifest hypertrophic cardiomyopathy, lipid accumulation in myocardium, degeneration of mitochondrial cristae, metabolic acidosis, and elevated plasma lactate levels. SCFI transmission pattern is consistent with autosomal recessive inheritance.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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