PolG1 - DNA polymerase gamma subunit 1

Enables 3'-5'-DNA exonuclease activity; DNA-directed DNA polymerase activity; and nucleotide binding activity. Involved in DNA replication proofreading and mitochondrion organization. Located in mitochondrial nucleoid. Part of gamma DNA polymerase complex. Used to study Alpers-Huttenlocher syndrome and mitochondrial metabolism disease. Human ortholog(s) of this gene implicated in several diseases, including mitochondrial DNA depletion syndrome (multiple); mitochondrial myopathy (multiple); neurodegenerative disease (multiple); ovarian disease (multiple); and sensory ataxic neuropathy, dysarthria, and ophthalmoparesis. Orthologous to human POLG (DNA polymerase gamma, catalytic subunit). [provided by Alliance of Genome Resources, Nov 2024]

NCBI Orthologs

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