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Protein

Mitochondrial inner membrane protease ATP23 homolog

Gene

ATP23

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi125Divalent metal cation; catalyticBy similarity1
Active sitei126PROSITE-ProRule annotation1
Metal bindingi129Divalent metal cation; catalyticBy similarity1

GO - Molecular functioni

  • DNA-dependent protein kinase activity Source: UniProtKB
  • metal ion binding Source: UniProtKB-KW
  • metalloendopeptidase activity Source: InterPro

GO - Biological processi

  • double-strand break repair via nonhomologous end joining Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

Metal-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000166896-MONOMER.

Protein family/group databases

MEROPSiM76.001.

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial inner membrane protease ATP23 homolog (EC:3.4.24.-)
Alternative name(s):
Ku70-binding protein 3
XRCC6-binding protein 1
Gene namesi
Name:ATP23Imported
Synonyms:KUB3, XRCC6BP1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:29452. ATP23.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Pathology & Biotechi

Organism-specific databases

DisGeNETi91419.
OpenTargetsiENSG00000166896.
ENSG00000262145.
PharmGKBiPA142670557.

Polymorphism and mutation databases

BioMutaiXRCC6BP1.
DMDMi224471894.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003303441 – 246Mitochondrial inner membrane protease ATP23 homologAdd BLAST246

Proteomic databases

EPDiQ9Y6H3.
MaxQBiQ9Y6H3.
PaxDbiQ9Y6H3.
PeptideAtlasiQ9Y6H3.
PRIDEiQ9Y6H3.

PTM databases

iPTMnetiQ9Y6H3.
PhosphoSitePlusiQ9Y6H3.

Expressioni

Gene expression databases

BgeeiENSG00000166896.
CleanExiHS_XRCC6BP1.
ExpressionAtlasiQ9Y6H3. baseline and differential.
GenevisibleiQ9Y6H3. HS.

Organism-specific databases

HPAiHPA035804.
HPA043204.

Interactioni

Subunit structurei

Interacts with XRCC6.1 Publication

Protein-protein interaction databases

BioGridi124830. 13 interactors.
STRINGi9606.ENSP00000300145.

Structurei

3D structure databases

ProteinModelPortaliQ9Y6H3.
SMRiQ9Y6H3.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the peptidase M76 family.Curated

Phylogenomic databases

eggNOGiKOG3314. Eukaryota.
ENOG4111IRG. LUCA.
GeneTreeiENSGT00390000010948.
HOGENOMiHOG000244058.
HOVERGENiHBG055037.
InParanoidiQ9Y6H3.
KOiK18156.
OMAiVDWFTNI.
OrthoDBiEOG091G0K0H.
PhylomeDBiQ9Y6H3.
TreeFamiTF327014.

Family and domain databases

InterProiIPR019165. Peptidase_M76_ATP23.
[Graphical view]
PfamiPF09768. Peptidase_M76. 1 hit.
[Graphical view]
PROSITEiPS00142. ZINC_PROTEASE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q9Y6H3-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGAPDERRR GPAAGEQLQQ QHVSCQVFPE RLAQGNPQQG FFSSFFTSNQ
60 70 80 90 100
KCQLRLLKTL ETNPYVKLLL DAMKHSGCAV NKDRHFSCED CNGNVSGGFD
110 120 130 140 150
ASTSQIVLCQ NNIHNQAHMN RVVTHELIHA FDHCRAHVDW FTNIRHLACS
160 170 180 190 200
EVRAANLSGD CSLVNEIFRL HFGLKQHHQT CVRDRATLSI LAVRNISKEV
210 220 230 240
AKKAVDEVFE SCFNDHEPFG RIPHNKTYAR YAHRDFENRD RYYSNI
Length:246
Mass (Da):28,081
Last modified:March 3, 2009 - v3
Checksum:iA9541CBEA299A35F
GO

Sequence cautioni

The sequence AAD31085 differs from that shown. Reason: Erroneous initiation.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05448948S → C.1 PublicationCorresponds to variant rs3751325dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF078164 mRNA. Translation: AAD31085.1. Different initiation.
AC084033 Genomic DNA. No translation available.
CH471054 Genomic DNA. Translation: EAW97091.1.
BC012776 mRNA. Translation: AAH12776.3.
BC115381 mRNA. Translation: AAI15382.1.
BC115382 mRNA. Translation: AAI15383.1.
CCDSiCCDS41802.1.
RefSeqiNP_001307337.1. NM_001320408.1.
NP_001307338.1. NM_001320409.1.
NP_001307339.1. NM_001320410.1.
NP_150592.1. NM_033276.3.
UniGeneiHs.61188.

Genome annotation databases

EnsembliENST00000300145; ENSP00000300145; ENSG00000166896.
ENST00000571490; ENSP00000460944; ENSG00000262145.
GeneIDi91419.
KEGGihsa:91419.
UCSCiuc001sqp.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF078164 mRNA. Translation: AAD31085.1. Different initiation.
AC084033 Genomic DNA. No translation available.
CH471054 Genomic DNA. Translation: EAW97091.1.
BC012776 mRNA. Translation: AAH12776.3.
BC115381 mRNA. Translation: AAI15382.1.
BC115382 mRNA. Translation: AAI15383.1.
CCDSiCCDS41802.1.
RefSeqiNP_001307337.1. NM_001320408.1.
NP_001307338.1. NM_001320409.1.
NP_001307339.1. NM_001320410.1.
NP_150592.1. NM_033276.3.
UniGeneiHs.61188.

3D structure databases

ProteinModelPortaliQ9Y6H3.
SMRiQ9Y6H3.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124830. 13 interactors.
STRINGi9606.ENSP00000300145.

Protein family/group databases

MEROPSiM76.001.

PTM databases

iPTMnetiQ9Y6H3.
PhosphoSitePlusiQ9Y6H3.

Polymorphism and mutation databases

BioMutaiXRCC6BP1.
DMDMi224471894.

Proteomic databases

EPDiQ9Y6H3.
MaxQBiQ9Y6H3.
PaxDbiQ9Y6H3.
PeptideAtlasiQ9Y6H3.
PRIDEiQ9Y6H3.

Protocols and materials databases

DNASUi91419.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000300145; ENSP00000300145; ENSG00000166896.
ENST00000571490; ENSP00000460944; ENSG00000262145.
GeneIDi91419.
KEGGihsa:91419.
UCSCiuc001sqp.4. human.

Organism-specific databases

CTDi91419.
DisGeNETi91419.
GeneCardsiXRCC6BP1.
H-InvDBHIX0010758.
HGNCiHGNC:29452. ATP23.
HPAiHPA035804.
HPA043204.
neXtProtiNX_Q9Y6H3.
OpenTargetsiENSG00000166896.
ENSG00000262145.
PharmGKBiPA142670557.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3314. Eukaryota.
ENOG4111IRG. LUCA.
GeneTreeiENSGT00390000010948.
HOGENOMiHOG000244058.
HOVERGENiHBG055037.
InParanoidiQ9Y6H3.
KOiK18156.
OMAiVDWFTNI.
OrthoDBiEOG091G0K0H.
PhylomeDBiQ9Y6H3.
TreeFamiTF327014.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000166896-MONOMER.

Miscellaneous databases

GenomeRNAii91419.
PROiQ9Y6H3.

Gene expression databases

BgeeiENSG00000166896.
CleanExiHS_XRCC6BP1.
ExpressionAtlasiQ9Y6H3. baseline and differential.
GenevisibleiQ9Y6H3. HS.

Family and domain databases

InterProiIPR019165. Peptidase_M76_ATP23.
[Graphical view]
PfamiPF09768. Peptidase_M76. 1 hit.
[Graphical view]
PROSITEiPS00142. ZINC_PROTEASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiATP23_HUMAN
AccessioniPrimary (citable) accession number: Q9Y6H3
Secondary accession number(s): Q1RLM4, Q96E81
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 29, 2008
Last sequence update: March 3, 2009
Last modified: November 2, 2016
This is version 103 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. Peptidase families
    Classification of peptidase families and list of entries
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.