ID   ATP23_HUMAN             Reviewed;         246 AA.
AC   Q9Y6H3; Q1RLM4; Q96E81;
DT   29-APR-2008, integrated into UniProtKB/Swiss-Prot.
DT   03-MAR-2009, sequence version 3.
DT   28-JAN-2026, entry version 152.
DE   RecName: Full=Mitochondrial inner membrane protease ATP23 homolog;
DE            EC=3.4.24.-;
DE   AltName: Full=Ku70-binding protein 3;
DE   AltName: Full=XRCC6-binding protein 1;
GN   Name=ATP23 {ECO:0000312|HGNC:HGNC:29452}; Synonyms=KUB3, XRCC6BP1;
OS   Homo sapiens (Human).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC   Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC   Homo.
OX   NCBI_TaxID=9606;
RN   [1]
RP   NUCLEOTIDE SEQUENCE [MRNA], VARIANT CYS-48, AND INTERACTION WITH XRCC6.
RC   TISSUE=Liver;
RX   PubMed=10219089; DOI=10.1093/nar/27.10.2165;
RA   Yang C.-R., Yeh S.-Y., Leskov K., Odegaard E., Hsu H.L., Chang C.,
RA   Kinsella T.J., Chen D.J., Boothman D.A.;
RT   "Isolation of Ku70-binding proteins (KUBs).";
RL   Nucleic Acids Res. 27:2165-2174(1999).
RN   [2]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RX   PubMed=16541075; DOI=10.1038/nature04569;
RA   Scherer S.E., Muzny D.M., Buhay C.J., Chen R., Cree A., Ding Y.,
RA   Dugan-Rocha S., Gill R., Gunaratne P., Harris R.A., Hawes A.C.,
RA   Hernandez J., Hodgson A.V., Hume J., Jackson A., Khan Z.M., Kovar-Smith C.,
RA   Lewis L.R., Lozado R.J., Metzker M.L., Milosavljevic A., Miner G.R.,
RA   Montgomery K.T., Morgan M.B., Nazareth L.V., Scott G., Sodergren E.,
RA   Song X.-Z., Steffen D., Lovering R.C., Wheeler D.A., Worley K.C., Yuan Y.,
RA   Zhang Z., Adams C.Q., Ansari-Lari M.A., Ayele M., Brown M.J., Chen G.,
RA   Chen Z., Clerc-Blankenburg K.P., Davis C., Delgado O., Dinh H.H.,
RA   Draper H., Gonzalez-Garay M.L., Havlak P., Jackson L.R., Jacob L.S.,
RA   Kelly S.H., Li L., Li Z., Liu J., Liu W., Lu J., Maheshwari M.,
RA   Nguyen B.-V., Okwuonu G.O., Pasternak S., Perez L.M., Plopper F.J.H.,
RA   Santibanez J., Shen H., Tabor P.E., Verduzco D., Waldron L., Wang Q.,
RA   Williams G.A., Zhang J., Zhou J., Allen C.C., Amin A.G., Anyalebechi V.,
RA   Bailey M., Barbaria J.A., Bimage K.E., Bryant N.P., Burch P.E.,
RA   Burkett C.E., Burrell K.L., Calderon E., Cardenas V., Carter K., Casias K.,
RA   Cavazos I., Cavazos S.R., Ceasar H., Chacko J., Chan S.N., Chavez D.,
RA   Christopoulos C., Chu J., Cockrell R., Cox C.D., Dang M., Dathorne S.R.,
RA   David R., Davis C.M., Davy-Carroll L., Deshazo D.R., Donlin J.E.,
RA   D'Souza L., Eaves K.A., Egan A., Emery-Cohen A.J., Escotto M., Flagg N.,
RA   Forbes L.D., Gabisi A.M., Garza M., Hamilton C., Henderson N.,
RA   Hernandez O., Hines S., Hogues M.E., Huang M., Idlebird D.G., Johnson R.,
RA   Jolivet A., Jones S., Kagan R., King L.M., Leal B., Lebow H., Lee S.,
RA   LeVan J.M., Lewis L.C., London P., Lorensuhewa L.M., Loulseged H.,
RA   Lovett D.A., Lucier A., Lucier R.L., Ma J., Madu R.C., Mapua P.,
RA   Martindale A.D., Martinez E., Massey E., Mawhiney S., Meador M.G.,
RA   Mendez S., Mercado C., Mercado I.C., Merritt C.E., Miner Z.L., Minja E.,
RA   Mitchell T., Mohabbat F., Mohabbat K., Montgomery B., Moore N., Morris S.,
RA   Munidasa M., Ngo R.N., Nguyen N.B., Nickerson E., Nwaokelemeh O.O.,
RA   Nwokenkwo S., Obregon M., Oguh M., Oragunye N., Oviedo R.J., Parish B.J.,
RA   Parker D.N., Parrish J., Parks K.L., Paul H.A., Payton B.A., Perez A.,
RA   Perrin W., Pickens A., Primus E.L., Pu L.-L., Puazo M., Quiles M.M.,
RA   Quiroz J.B., Rabata D., Reeves K., Ruiz S.J., Shao H., Sisson I.,
RA   Sonaike T., Sorelle R.P., Sutton A.E., Svatek A.F., Svetz L.A.,
RA   Tamerisa K.S., Taylor T.R., Teague B., Thomas N., Thorn R.D., Trejos Z.Y.,
RA   Trevino B.K., Ukegbu O.N., Urban J.B., Vasquez L.I., Vera V.A.,
RA   Villasana D.M., Wang L., Ward-Moore S., Warren J.T., Wei X., White F.,
RA   Williamson A.L., Wleczyk R., Wooden H.S., Wooden S.H., Yen J., Yoon L.,
RA   Yoon V., Zorrilla S.E., Nelson D., Kucherlapati R., Weinstock G.,
RA   Gibbs R.A.;
RT   "The finished DNA sequence of human chromosome 12.";
RL   Nature 440:346-351(2006).
RN   [3]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RA   Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M.,
RA   Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J.,
RA   Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S.,
RA   Turner R., Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H.,
RA   Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K.,
RA   Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D.,
RA   Hunkapiller M.W., Myers E.W., Venter J.C.;
RL   Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases.
RN   [4]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC   TISSUE=Lung;
RX   PubMed=15489334; DOI=10.1101/gr.2596504;
RG   The MGC Project Team;
RT   "The status, quality, and expansion of the NIH full-length cDNA project:
RT   the Mammalian Gene Collection (MGC).";
RL   Genome Res. 14:2121-2127(2004).
CC   -!- SUBUNIT: Interacts with XRCC6. {ECO:0000269|PubMed:10219089}.
CC   -!- SIMILARITY: Belongs to the peptidase M76 family. {ECO:0000305}.
CC   -!- SEQUENCE CAUTION:
CC       Sequence=AAD31085.1; Type=Erroneous initiation; Evidence={ECO:0000305};
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DR   EMBL; AF078164; AAD31085.1; ALT_INIT; mRNA.
DR   EMBL; AC084033; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR   EMBL; CH471054; EAW97091.1; -; Genomic_DNA.
DR   EMBL; BC012776; AAH12776.3; -; mRNA.
DR   EMBL; BC115381; AAI15382.1; -; mRNA.
DR   EMBL; BC115382; AAI15383.1; -; mRNA.
DR   CCDS; CCDS41802.1; -.
DR   RefSeq; NP_001307337.1; NM_001320408.1.
DR   RefSeq; NP_001307338.1; NM_001320409.1.
DR   RefSeq; NP_001307339.1; NM_001320410.1.
DR   RefSeq; NP_150592.1; NM_033276.4.
DR   AlphaFoldDB; Q9Y6H3; -.
DR   BioGRID; 124830; 63.
DR   FunCoup; Q9Y6H3; 1999.
DR   STRING; 9606.ENSP00000300145; -.
DR   MEROPS; M76.001; -.
DR   GlyGen; Q9Y6H3; 1 site, 1 O-linked glycan (1 site).
DR   iPTMnet; Q9Y6H3; -.
DR   PhosphoSitePlus; Q9Y6H3; -.
DR   BioMuta; ATP23; -.
DR   DMDM; 224471894; -.
DR   jPOST; Q9Y6H3; -.
DR   MassIVE; Q9Y6H3; -.
DR   PaxDb; 9606-ENSP00000300145; -.
DR   PeptideAtlas; Q9Y6H3; -.
DR   ProteomicsDB; 86679; -.
DR   Pumba; Q9Y6H3; -.
DR   Antibodypedia; 28994; 101 antibodies from 17 providers.
DR   DNASU; 91419; -.
DR   Ensembl; ENST00000300145.4; ENSP00000300145.3; ENSG00000166896.10.
DR   Ensembl; ENST00000571490.1; ENSP00000460944.1; ENSG00000262145.5.
DR   GeneID; 91419; -.
DR   KEGG; hsa:91419; -.
DR   MANE-Select; ENST00000300145.4; ENSP00000300145.3; NM_033276.4; NP_150592.1.
DR   UCSC; uc001sqp.4; human.
DR   AGR; HGNC:29452; -.
DR   ClinPGx; PA142670557; -.
DR   CTD; 91419; -.
DR   DisGeNET; 91419; -.
DR   GeneCards; ATP23; -.
DR   HGNC; HGNC:29452; ATP23.
DR   HPA; ENSG00000166896; Tissue enhanced (testis).
DR   MIM; 619760; gene.
DR   OpenTargets; ENSG00000166896; -.
DR   VEuPathDB; HostDB:ENSG00000166896; -.
DR   eggNOG; KOG3314; Eukaryota.
DR   GeneTree; ENSGT00390000010948; -.
DR   HOGENOM; CLU_079125_1_0_1; -.
DR   InParanoid; Q9Y6H3; -.
DR   OMA; KRHHQTC; -.
DR   OrthoDB; 285308at2759; -.
DR   PAN-GO; Q9Y6H3; 3 GO annotations based on evolutionary models.
DR   PhylomeDB; Q9Y6H3; -.
DR   PathwayCommons; Q9Y6H3; -.
DR   SignaLink; Q9Y6H3; -.
DR   Agora; ENSG00000166896; -.
DR   BioGRID-ORCS; 91419; 60 hits in 1145 CRISPR screens.
DR   ChiTaRS; ATP23; human.
DR   GenomeRNAi; 91419; -.
DR   Pharos; Q9Y6H3; Tbio.
DR   PRO; PR:Q9Y6H3; -.
DR   Proteomes; UP000005640; Chromosome 12.
DR   RNAct; Q9Y6H3; protein.
DR   Bgee; ENSG00000166896; Expressed in right testis and 102 other cell types or tissues.
DR   ExpressionAtlas; Q9Y6H3; baseline and differential.
DR   GO; GO:0030054; C:cell junction; IDA:HPA.
DR   GO; GO:0005829; C:cytosol; IDA:HPA.
DR   GO; GO:0005958; C:DNA-dependent protein kinase-DNA ligase 4 complex; NAS:UniProtKB.
DR   GO; GO:0005739; C:mitochondrion; HTP:FlyBase.
DR   GO; GO:0005886; C:plasma membrane; IDA:HPA.
DR   GO; GO:0004677; F:DNA-dependent protein kinase activity; TAS:UniProtKB.
DR   GO; GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
DR   GO; GO:0004222; F:metalloendopeptidase activity; IEA:InterPro.
DR   GO; GO:0006303; P:double-strand break repair via nonhomologous end joining; TAS:UniProtKB.
DR   GO; GO:0034982; P:mitochondrial protein processing; IBA:GO_Central.
DR   GO; GO:0033615; P:mitochondrial proton-transporting ATP synthase complex assembly; IBA:GO_Central.
DR   InterPro; IPR019165; Peptidase_M76_ATP23.
DR   PANTHER; PTHR21711; MITOCHONDRIAL INNER MEMBRANE PROTEASE; 1.
DR   PANTHER; PTHR21711:SF0; MITOCHONDRIAL INNER MEMBRANE PROTEASE ATP23 HOMOLOG; 1.
DR   Pfam; PF09768; Peptidase_M76; 1.
DR   PROSITE; PS00142; ZINC_PROTEASE; 1.
PE   1: Evidence at protein level;
KW   Hydrolase; Metal-binding; Metalloprotease; Protease;
KW   Proteomics identification; Reference proteome.
FT   CHAIN           1..246
FT                   /note="Mitochondrial inner membrane protease ATP23 homolog"
FT                   /id="PRO_0000330344"
FT   ACT_SITE        126
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU10095"
FT   BINDING         125
FT                   /ligand="a divalent metal cation"
FT                   /ligand_id="ChEBI:CHEBI:60240"
FT                   /ligand_note="catalytic"
FT                   /evidence="ECO:0000250"
FT   BINDING         129
FT                   /ligand="a divalent metal cation"
FT                   /ligand_id="ChEBI:CHEBI:60240"
FT                   /ligand_note="catalytic"
FT                   /evidence="ECO:0000250"
FT   VARIANT         48
FT                   /note="S -> C (in dbSNP:rs3751325)"
FT                   /evidence="ECO:0000269|PubMed:10219089"
FT                   /id="VAR_054489"
SQ   SEQUENCE   246 AA;  28081 MW;  A9541CBEA299A35F CRC64;
     MAGAPDERRR GPAAGEQLQQ QHVSCQVFPE RLAQGNPQQG FFSSFFTSNQ KCQLRLLKTL
     ETNPYVKLLL DAMKHSGCAV NKDRHFSCED CNGNVSGGFD ASTSQIVLCQ NNIHNQAHMN
     RVVTHELIHA FDHCRAHVDW FTNIRHLACS EVRAANLSGD CSLVNEIFRL HFGLKQHHQT
     CVRDRATLSI LAVRNISKEV AKKAVDEVFE SCFNDHEPFG RIPHNKTYAR YAHRDFENRD
     RYYSNI
//