PPA2 Antibody, FITC conjugated

Code CSB-PA867137LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) PPA2 Polyclonal antibody
Uniprot No.
Target Names
PPA2
Alternative Names
HSPC124 antibody; Inorganic pyrophosphatase 2 antibody; Inorganic pyrophosphatase 2, mitochondrial antibody; IPYR2_HUMAN antibody; mitochondrial antibody; Ppa2 antibody; PPase 2 antibody; PPase2 antibody; Pyrophosphatase (inorganic) 2 antibody; Pyrophosphatase SID6 306 antibody; Pyrophosphatase SID6-306 antibody; Pyrophosphate phospho-hydrolase 2 antibody; SID6 306 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Inorganic pyrophosphatase 2, mitochondrial protein (180-298AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Hydrolyzes inorganic pyrophosphate. This activity is essential for correct regulation of mitochondrial membrane potential, and mitochondrial organization and function.
Gene References into Functions
  1. findings confirm the pathogenicity of PPA2 mutations and suggest that PPA2 is a cardiomyopathy-associated protein, which has a greater physiological importance in mitochondrial function than previously recognized PMID: 27523597
  2. data demonstrate that PPA2 is an essential gene in yeast and that biallelic mutations in PPA2 cause a mitochondrial disease leading to sudden cardiac arrest in infants PMID: 27523598
  3. Single nucleotide polymorphisms in PPA2 is associated with response to antipsychotic agents in schizophrenia. PMID: 23241943
  4. We think that PP2A can be one of the key components to regulate the fusion of various endocytotic compartments and /or the trafficking along the microtubules. PMID: 19067239
  5. No pathogenic mutations were identified in the PPA2 gene in patients with mitochondrial DNA depletion syndromes (MDS). PMID: 16300924

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Involvement in disease
Sudden cardiac failure, alcohol-induced (SCFAI); Sudden cardiac failure, infantile (SCFI)
Subcellular Location
Mitochondrion.
Protein Families
PPase family
Tissue Specificity
Detected in brain, gastric carcinoma, lung, ovary, skeletal muscle, umbilical cord blood and a cell line derived from kidney proximal tubule epithelium.
Database Links

HGNC: 28883

OMIM: 609988

KEGG: hsa:27068

STRING: 9606.ENSP00000343885

UniGene: Hs.654957

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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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