DNA polymerase subunit gamma-2 Recombinant Protein | POLG2 recombinant protein

Recombinant Human DNA polymerase subunit gamma-2, mitochondrial

Cat. Num. AAA1486806

• Gene Names: POLG2; HP55; POLB; PEOA4; POLGB; MTPOLB; POLG-BETA
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: DNA polymerase subunit gamma-2; Recombinant Human DNA polymerase subunit gamma-2; mitochondrial; DNA polymerase gamma accessory 55 kDa subunit; p55; Mitochondrial DNA polymerase accessory subunit; MtPolB; PolG-beta; POLG2 recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 1-485aa; Full Length
• Sequence: MRSRVAVRACHKVCRCLLSGFGGRVDAGQPELLTERSSPKGGHVKSHAELEGNGEHPEAPGSGEGSEALLEICQRRHFLSGSKQQLSRDSLLSGCHPGFGPLGVELRKNLAAEWWTSVVVFREQVFPVDALHHKPGPLLPGDSAFRLVSAETLREILQDKELSKEQLVAFLENVLKTSGKLRENLLHGALEHYVNCLDLVNKRLPYGLAQIGVCFHPVFDTKQIRNGVKSIGEKTEASLVWFTPPRTSNQWLDFWLRHRLQWWRKFAMSPSNFSSSDCQDEEGRKGNKLYYNFPWGKELIETLWNLGDHELLHMYPGNVSKLHGRDGRKNVVPCVLSVNGDLDRGMLAYLYDSFQLTENSFTRKKNLHRKVLKLHPCLAPIKVALDVGRGPTLELRQVCQGLFNELLENGISVWPGYLETMQSSLEQLYSKYDEMSILFTVLVTETTLENGLIHLRSRDTTMKEMMHISKLKDFLIKYISSAKNV
• Sequence Length: 485

• Preparation and Storage: Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

SDS-PAGE

Related Product Information for POLG2 recombinant protein

Mitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA.

Product Categories/Family for POLG2 recombinant protein

Transcription

References

The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance.Lim S.E., Longley M.J., Copeland W.C.J. Biol. Chem. 274:38197-38203(1999) Protein sequences conserved in prokaryotic aminoacyl-tRNA synthetases are important for the activity of the processivity factor of human mitochondrial DNA polymerase.Carrodeguas J.A., Bogenhagen D.F.Nucleic Acids Res. 28:1237-1244(2000) Human mitochondrial DNA polymerase holoenzyme reconstitution and characterization.Johnson A.A., Tsai Y.-C., Graves S.W., Johnson K.A.Biochemistry 39:1702-1708(2000) Accessory subunit of mitochondrial DNA polymerase from Drosophila embryos. Cloning, molecular analysis, and association in the native enzyme.Wang Y., Farr C.L., Kaguni L.S.J. Biol. Chem. 272:13640-13646(1997) Mutant POLG2 disrupts DNA polymerase gamma subunits and causes progressive external ophthalmoplegia.Longley M.J., Clark S., Man C.Y.W., Hudson G., Durham S.E., Taylor R.W., Nightingale S., Turnbull D.M., Copeland W.C., Chinnery P.F.Am. J. Hum. Genet. 78:1026-1034(2006) Progressive external ophthalmoplegia and vision and hearing loss in a patient with mutations in POLG2 and OPA1.Ferraris S., Clark S., Garelli E., Davidzon G., Moore S.A., Kardon R.H., Bienstock R.J., Longley M.J., Mancuso M., Gutierrez Rios P., Hirano M., Copeland W.C., DiMauro S.Arch. Neurol. 65:125-131(2008)

NCBI and Uniprot Product Information

• NCBI GI #: 70887790
• NCBI GeneID: 11232
• NCBI Accession #: NP_009146.2
• NCBI GenBank Nucleotide #: NM_007215.3
• UniProt Accession #: Q9UHN1; O00419; Q0IJ81; Q96GW2; Q9UK35; Q9UK94
• Molecular Weight: 70.9 kDa
• NCBI Official Full Name: DNA polymerase subunit gamma-2, mitochondrial
• NCBI Official Synonym Full Names: polymerase (DNA directed), gamma 2, accessory subunit
• NCBI Official Symbol: POLG2
• NCBI Official Synonym Symbols: HP55; POLB; PEOA4; POLGB; MTPOLB; POLG-BETA
• NCBI Protein Information: DNA polymerase subunit gamma-2, mitochondrial
• UniProt Protein Name: DNA polymerase subunit gamma-2, mitochondrial
• Protein Family: DNA polymerase
• UniProt Gene Name: POLG2
• UniProt Synonym Gene Names: MTPOLB; p55
• UniProt Entry Name: DPOG2_HUMAN

NCBI Description

This gene encodes the processivity subunit of the mitochondrial DNA polymerase gamma. The encoded protein forms a heterotrimer containing one catalytic subunit and two processivity subunits. This protein enhances DNA binding and promotes processive DNA synthesis. Mutations in this gene result in autosomal dominant progressive external ophthalmoplegia with mitochondrial DNA deletions.[provided by RefSeq, Sep 2009]

Uniprot Description

POLG2: Mitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA. Defects in POLG2 are the cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 4 (PEOA4). Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged- red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism.

Protein type: EC 2.7.7.7; Mitochondrial; Transferase; DNA repair, damage; DNA replication

Chromosomal Location of Human Ortholog: 17q

Cellular Component: mitochondrial matrix

Molecular Function: DNA binding; DNA-directed DNA polymerase activity; glycine-tRNA ligase activity; identical protein binding; protein binding

Biological Process: DNA repair; DNA replication; DNA-dependent DNA replication; glycyl-tRNA aminoacylation; in utero embryonic development; mitochondrial DNA metabolic process; mitochondrion organization and biogenesis; organelle organization and biogenesis

Disease: Progressive External Ophthalmoplegia With Mitochondrial Dna Deletions, Autosomal Dominant, 4