MitoProteome Database

MT000685

UniProt Annotations

Entry Information
Gene NamealkB, alkylation repair homolog 1 (E. coli)
Protein EntryALKB1_HUMAN
UniProt IDQ13686
SpeciesHuman
Comments
Comment typeDescription
Catalytic Activity DNA-base-CH(3) + 2-oxoglutarate + O(2) = DNA- base + formaldehyde + succinate + CO(2).
Catalytic Activity The C-O-P bond 3' to the apurinic or apyrimidinic site in DNA is broken by a beta-elimination reaction, leaving a 3'-terminal unsaturated sugar and a product with a terminal 5'-phosphate.
Caution Detected in cytoplasm and nucleus When expressed as fusion protein with an N-terminal tag, or when the first 26 N- terminal residues are removed (PubMed:17979886 and PubMed:18603530). The endogenous, unmodified protein localizes to mitochondria (PubMed:18603530). {ECO:0000305|PubMed:18603530}.
Cofactor Name=Fe(2+); Xref=ChEBI:CHEBI:29033; Evidence={ECO:0000255|PROSITE-ProRule:PRU00805, ECO:0000269|PubMed:18603530}; Note=Binds 1 Fe(2+) ion per subunit. {ECO:0000255|PROSITE- ProRule:PRU00805, ECO:0000269|PubMed:18603530};
Function Dioxygenase that repairs alkylated single-stranded DNA and RNA containing 3-methylcytosine by oxidative demethylation. Requires molecular oxygen, alpha-ketoglutarate and iron. May have a role in placental trophoblast lineage differentiation (By similarity). Has DNA lyase activity and introduces double-stranded breaks at abasic sites. Cleaves both single-stranded DNA and double-stranded DNA at abasic sites, with the greatest activity towards double-stranded DNA with two abasic sites. DNA lyase activity does not require alpha-ketoglutarate and iron. {ECO:0000250, ECO:0000269|PubMed:18603530, ECO:0000269|PubMed:19959401}.
Sequence Caution Sequence=CAA63047.1; Type=Frameshift; Positions=Several; Evidence={ECO:0000305};
Similarity Belongs to the alkB family. {ECO:0000305}.
Similarity Contains 1 Fe2OG dioxygenase domain. {ECO:0000255|PROSITE-ProRule:PRU00805}.
Subcellular Location Mitochondrion {ECO:0000269|PubMed:17979886, ECO:0000269|PubMed:18603530}. Nucleus {ECO:0000250}. Note=Mainly localizes in euchromatin, largely excluded from heterochromatin and nucleoli. {ECO:0000250}.
Subunit Monomer. Interacts with DNAJB6 (By similarity). {ECO:0000250}.
Tissue Specificity Ubiquitous. {ECO:0000269|PubMed:17979886, ECO:0000269|PubMed:18603530}.