Recombinant Human Inorganic pyrophosphatase 2, mitochondrial (PPA2)

Code CSB-YP867137HU
MSDS
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Source Yeast
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Code CSB-EP867137HU-B
MSDS
Size Pls inquire
Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP867137HU
MSDS
Size Pls inquire
Source Baculovirus
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Code CSB-MP867137HU
MSDS
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
PPA2
Uniprot No.
Alternative Names
HSPC124; Inorganic pyrophosphatase 2; Inorganic pyrophosphatase 2, mitochondrial; IPYR2_HUMAN; mitochondrial; Ppa2; PPase 2; PPase2; Pyrophosphatase (inorganic) 2; Pyrophosphatase SID6 306 ; Pyrophosphatase SID6-306; Pyrophosphate phospho-hydrolase 2; SID6 306
Species
Homo sapiens (Human)
Expression Region
33-334
Target Protein Sequence
ALYHTEER GQPCSQNYRL FFKNVTGHYI SPFHDIPLKV NSKEENGIPM KKARNDEYEN LFNMIVEIPR WTNAKMEIAT KEPMNPIKQY VKDGKLRYVA NIFPYKGYIW NYGTLPQTWE DPHEKDKSTN CFGDNDPIDV CEIGSKILSC GEVIHVKILG ILALIDEGET DWKLIAINAN DPEASKFHDI DDVKKFKPGY LEATLNWFRL YKVPDGKPEN QFAFNGEFKN KAFALEVIKS THQCWKALLM KKCNGGAINC TNVQISDSPF RCTQEEARSL VESVSSSPNK ESNEEEQVWH FLGK
Protein Length
Full Length of Mature Protein
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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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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