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NameDNA polymerase subunit gamma-2, mitochondrial
Synonyms
  • 2.7.7.7
  • DNA polymerase gamma accessory 55 kDa subunit
  • Mitochondrial DNA polymerase accessory subunit
  • MTPOLB
  • p55
  • PolG-beta
Gene NamePOLG2
OrganismHuman
Amino acid sequence
>lcl|BSEQ0017587|DNA polymerase subunit gamma-2, mitochondrial
MRSRVAVRACHKVCRCLLSGFGGRVDAGQPELLTERSSPKGGHVKSHAELEGNGEHPEAP
GSGEGSEALLEICQRRHFLSGSKQQLSRDSLLSGCHPGFGPLGVELRKNLAAEWWTSVVV
FREQVFPVDALHHKPGPLLPGDSAFRLVSAETLREILQDKELSKEQLVAFLENVLKTSGK
LRENLLHGALEHYVNCLDLVNKRLPYGLAQIGVCFHPVFDTKQIRNGVKSIGEKTEASLV
WFTPPRTSNQWLDFWLRHRLQWWRKFAMSPSNFSSSDCQDEEGRKGNKLYYNFPWGKELI
ETLWNLGDHELLHMYPGNVSKLHGRDGRKNVVPCVLSVNGDLDRGMLAYLYDSFQLTENS
FTRKKNLHRKVLKLHPCLAPIKVALDVGRGPTLELRQVCQGLFNELLENGISVWPGYLET
MQSSLEQLYSKYDEMSILFTVLVTETTLENGLIHLRSRDTTMKEMMHISKLKDFLIKYIS
SAKNV
Number of residues485
Molecular Weight54910.67
Theoretical pINot Available
GO Classification
Functions
  • DNA binding
  • DNA-directed DNA polymerase activity
Processes
  • DNA replication
  • DNA-dependent DNA replication
  • DNA repair
  • in utero embryonic development
  • respiratory electron transport chain
  • organelle organization
  • DNA biosynthetic process
  • mitochondrion organization
  • mitochondrion morphogenesis
  • mitochondrial DNA replication
Components
  • mitochondrial matrix
  • extracellular exosome
  • mitochondrial nucleoid
  • mitochondrial chromosome
General FunctionDna-directed dna polymerase activity
Specific FunctionMitochondrial polymerase processivity subunit. Stimulates the polymerase and exonuclease activities, and increases the processivity of the enzyme. Binds to ss-DNA.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
UniProtKB IDQ9UHN1
UniProtKB Entry NameDPOG2_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0017588|DNA polymerase subunit gamma-2, mitochondrial (POLG2)
ATGCGCTCTCGTGTAGCCGTCAGGGCCTGCCATAAGGTCTGCAGGTGCCTGTTGTCTGGG
TTTGGGGGTCGAGTAGATGCGGGGCAGCCGGAGCTGTTGACGGAAAGGAGTAGCCCCAAA
GGAGGGCATGTGAAGTCGCACGCGGAGCTCGAGGGGAACGGCGAGCACCCAGAAGCCCCC
GGGTCTGGAGAGGGAAGCGAGGCGCTGTTAGAGATCTGTCAGAGAAGGCATTTCCTAAGT
GGAAGCAAGCAGCAGCTTAGCCGGGATTCTCTTCTGAGTGGGTGCCACCCCGGCTTCGGA
CCCTTGGGCGTAGAGTTGCGGAAGAACCTGGCCGCAGAATGGTGGACCTCGGTGGTGGTG
TTCAGGGAGCAGGTATTCCCGGTGGACGCCCTCCACCACAAACCAGGCCCTTTGCTACCC
GGGGACAGTGCCTTCAGGTTAGTTTCTGCAGAAACTCTACGCGAAATCTTGCAAGACAAA
GAGCTGAGTAAGGAACAGCTAGTAGCATTTCTTGAGAACGTATTAAAAACTTCTGGGAAA
CTACGGGAGAACCTTCTTCACGGTGCCTTGGAACACTATGTTAATTGCCTGGATCTGGTA
AACAAGAGGCTACCTTATGGCCTTGCTCAGATTGGAGTGTGTTTTCATCCTGTTTTTGAC
ACTAAGCAGATACGAAATGGTGTTAAAAGTATTGGTGAGAAGACTGAAGCTTCGTTAGTA
TGGTTTACTCCTCCGAGAACTTCAAACCAGTGGCTTGATTTCTGGTTACGTCATCGACTC
CAGTGGTGGAGAAAGTTTGCCATGAGTCCATCTAACTTCAGCAGCAGTGACTGTCAGGAT
GAAGAAGGCCGGAAAGGAAACAAACTTTACTACAATTTTCCCTGGGGAAAGGAGTTAATA
GAAACCCTGTGGAACCTAGGAGATCACGAACTTTTACACATGTATCCTGGCAATGTGTCT
AAATTACATGGCCGAGATGGACGAAAAAATGTGGTTCCTTGTGTTCTCTCTGTAAATGGG
GACCTAGACCGAGGCATGCTGGCCTACCTCTATGATTCTTTCCAGCTGACAGAGAACTCC
TTTACAAGAAAGAAAAATCTTCATAGAAAGGTACTTAAACTTCACCCTTGTTTAGCCCCT
ATTAAGGTTGCTTTGGATGTAGGAAGAGGCCCCACATTGGAACTAAGACAGGTTTGTCAA
GGGCTATTTAATGAGTTACTAGAAAATGGGATTTCTGTGTGGCCTGGTTATTTGGAAACT
ATGCAGTCCTCATTGGAACAACTTTATTCGAAGTATGATGAAATGAGTATTCTCTTCACA
GTTTTGGTTACTGAAACTACTTTGGAGAATGGATTAATACATCTGAGAAGCAGAGACACC
ACAATGAAGGAAATGATGCATATATCCAAATTAAAAGACTTTTTGATTAAGTATATATCA
TCAGCTAAGAATGTATAG
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:9180
Chromosome Location17
LocusNot Available
References
  1. Lim SE, Longley MJ, Copeland WC: The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance. J Biol Chem. 1999 Dec 31;274(53):38197-203. 10608893
  2. Carrodeguas JA, Bogenhagen DF: Protein sequences conserved in prokaryotic aminoacyl-tRNA synthetases are important for the activity of the processivity factor of human mitochondrial DNA polymerase. Nucleic Acids Res. 2000 Mar 1;28(5):1237-44. 10666468
  3. Johnson AA, Tsai Yc, Graves SW, Johnson KA: Human mitochondrial DNA polymerase holoenzyme: reconstitution and characterization. Biochemistry. 2000 Feb 22;39(7):1702-8. 10677218
  4. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. 15489334
  5. Wang Y, Farr CL, Kaguni LS: Accessory subunit of mitochondrial DNA polymerase from Drosophila embryos. Cloning, molecular analysis, and association in the native enzyme. J Biol Chem. 1997 May 23;272(21):13640-6. 9153213
  6. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. 25944712
  7. Longley MJ, Clark S, Yu Wai Man C, Hudson G, Durham SE, Taylor RW, Nightingale S, Turnbull DM, Copeland WC, Chinnery PF: Mutant POLG2 disrupts DNA polymerase gamma subunits and causes progressive external ophthalmoplegia. Am J Hum Genet. 2006 Jun;78(6):1026-34. Epub 2006 May 4. 16685652
  8. Ferraris S, Clark S, Garelli E, Davidzon G, Moore SA, Kardon RH, Bienstock RJ, Longley MJ, Mancuso M, Gutierrez Rios P, Hirano M, Copeland WC, DiMauro S: Progressive external ophthalmoplegia and vision and hearing loss in a patient with mutations in POLG2 and OPA1. Arch Neurol. 2008 Jan;65(1):125-31. doi: 10.1001/archneurol.2007.9. 18195150