Sequence of this protein is as follows: HPVFDTKQIR NGVKSIGEKT EASLVWFTPP RTSNQWLDFW LRHRLQWWRK FAMSPSNFSS SDCQDEEGRK GNKLYYNFPW GKELIETLWN LGDHELLHMY PGNVSKLHGR DGRKNVVPCV LSVNGDLDRG MLAYLYDSFQ LTENSFTRKK NLHRKVLKLH PCLAPIKVAL DVGRGPTLEL RQVCQGLFNE LLENGISVWP GYLETMQSSL EQLYSKYDEM SILFTVLVTE TTLENGLIHL RSRDTTMKEM MHISKLKDFL IKYISSAKNV
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.
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Protein Aliases: DNA polymerase gamma; DNA polymerase gamma accessory 55 kDa subunit; DNA polymerase gamma subunit 1 (Mitochondrial DNA polymerase catalytic subunit) (PolG-alpha); DNA polymerase subunit gamma-1; DNA polymerase subunit gamma-2, mitochondrial; DNA-directed DNA polymerase gamma; Mitochondrial DNA polymerase accessory subunit; Mitochondrial DNA polymerase catalytic subunit; mitochondrial DNA polymerase gamma; mitochondrial DNA polymerase gamma catalytic subunit; mitochondrial DNA polymerase subunit gamma-2; mitochondrial DNA polymerase, accessory subunit; mitochondrial DNA polymerase-gamma; mitochondrial polymerase gamma; mitochondrial polymerase gamma 2; MtPolB; p55; Pol gamma; PolG-alpha; PolG-beta; polymerase (DNA directed), gamma; polymerase (DNA directed), gamma 2, accessory subunit; polymerase (DNA) gamma 2, accessory subunit; polymerase (DNA) gamma, catalytic subunit; polymerase, gamma; polymerase, gamma 2; truncated mitochondrial DNA polymerase gamma catalytic subunit
Gene Aliases: AA409516; HP55; MDP1; Mip1; MIRAS; MTDPS4A; MTDPS4B; MTPOLB; PEO; PEOA4; POLB; POLG; POLG-BETA; POLG1; POLG2; POLGA; POLGB; SANDO; SCAE
UniProt ID: (Human) P54098, (Human) Q9UHN1, (Rat) Q9QYV8, (Mouse) Q9QZM2
Entrez Gene ID: (Human) 5428, (Human) 11232, (Mouse) 18975, (Rat) 85472, (Rat) 303612, (Mouse) 50776
Molecular Function:
DNA binding protein
DNA-directed DNA polymerase
exodeoxyribonuclease
nuclease
nucleic acid binding
nucleotidyltransferase
transferase
aminoacyl-tRNA synthetase
ligase
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