NP_009146.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
54,911 Da
NCBI Official Full Name
DNA polymerase subunit gamma-2, mitochondrial
NCBI Official Synonym Full Names
polymerase (DNA directed), gamma 2, accessory subunit
NCBI Official Synonym Symbols
HP55; POLB; PEOA4; POLGB; MTPOLB; POLG-BETA [Similar Products]
NCBI Protein Information
DNA polymerase subunit gamma-2, mitochondrial
UniProt Protein Name
DNA polymerase subunit gamma-2, mitochondrial
UniProt Synonym Protein Names
DNA polymerase gamma accessory 55 kDa subunit; p55; Mitochondrial DNA polymerase accessory subunit; MtPolB; PolG-beta
UniProt Synonym Gene Names
UniProt Entry Name
DPOG2_HUMAN
NCBI Summary for POLG2
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 Comments for POLG2
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: Mitochondrial; EC 2.7.7.7; Transferase; DNA replication; DNA repair, damage
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
Research Articles on POLG2
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Products associated with anti-POLG2 antibody
Pathways associated with anti-POLG2 antibody
Diseases associated with anti-POLG2 antibody
Organs/Tissues associated with anti-POLG2 antibody
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