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Protein

Centromere protein X

Gene

CENPX

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

Functioni

DNA-binding component of the Fanconi anemia (FA) core complex. Required for the normal activation of the FA pathway, leading to monoubiquitination of the FANCI-FANCD2 complex in response to DNA damage, cellular resistance to DNA cross-linking drugs, and prevention of chromosomal breakage (PubMed:20347428, PubMed:20347429). In complex with CENPS (MHF heterodimer), crucial cofactor for FANCM in both binding and ATP-dependent remodeling of DNA. Stabilizes FANCM. In complex with CENPS and FANCM (but not other FANC proteins), rapidly recruited to blocked forks and promotes gene conversion at blocked replication forks (PubMed:20347428, PubMed:20347429). In complex with CENPS, CENPT and CENPW (CENP-T-W-S-X heterotetramer), involved in the formation of a functional kinetochore outer plate, which is essential for kinetochore-microtubule attachment and faithful mitotic progression (PubMed:19620631). As a component of MHF and CENP-T-W-S-X complexes, binds DNA and bends it to form a nucleosome-like structure (PubMed:20347428, PubMed:20347429). DNA-binding function is fulfilled in the presence of CENPS, with the following preference for DNA substates: Holliday junction > double-stranded > splay arm > single-stranded. Does not bind DNA on its own (PubMed:20347429).3 Publications

GO - Molecular functioni

  • DNA binding Source: UniProtKB

GO - Biological processi

  • cell division Source: UniProtKB-KW
  • CENP-A containing nucleosome assembly Source: Reactome
  • interstrand cross-link repair Source: Reactome
  • kinetochore assembly Source: InterPro
  • mitotic nuclear division Source: UniProtKB-KW
  • positive regulation of protein ubiquitination Source: UniProtKB
  • replication fork processing Source: UniProtKB
  • resolution of meiotic recombination intermediates Source: UniProtKB

Keywordsi

Molecular functionDNA-binding
Biological processCell cycle, Cell division, DNA damage, DNA repair, Mitosis

Enzyme and pathway databases

ReactomeiR-HSA-606279. Deposition of new CENPA-containing nucleosomes at the centromere.
R-HSA-6783310. Fanconi Anemia Pathway.
SIGNORiA8MT69.

Names & Taxonomyi

Protein namesi
Recommended name:
Centromere protein X
Short name:
CENP-X
Alternative name(s):
FANCM-associated histone fold protein 21 Publication
FANCM-interacting histone fold protein 21 Publication
Fanconi anemia-associated polypeptide of 10 kDa
Retinoic acid-inducible gene D9 protein homolog
Stimulated by retinoic acid gene 13 protein homolog
Gene namesi
Name:CENPX
Synonyms:FAAP10, MHF22 Publications, STRA13
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

HGNCiHGNC:11422. CENPX.

Subcellular locationi

GO - Cellular componenti

  • condensed chromosome kinetochore Source: UniProtKB-SubCell
  • FANCM-MHF complex Source: UniProtKB
  • Fanconi anaemia nuclear complex Source: UniProtKB
  • nucleoplasm Source: Reactome

Keywords - Cellular componenti

Centromere, Chromosome, Kinetochore, Nucleus

Pathology & Biotechi

Keywords - Diseasei

Fanconi anemia

Organism-specific databases

DisGeNETi201254.
OpenTargetsiENSG00000169689.
PharmGKBiPA36223.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003371801 – 81Centromere protein XAdd BLAST81

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiA8MT69.
MaxQBiA8MT69.
PRIDEiA8MT69.
TopDownProteomicsiA8MT69-1. [A8MT69-1]
A8MT69-2. [A8MT69-2]

PTM databases

iPTMnetiA8MT69.
PhosphoSitePlusiA8MT69.

Expressioni

Developmental stagei

Expression varies across the cell cycle, with highest levels in S phase (at protein level). No statistically significant changes at the transcript level.1 Publication

Gene expression databases

BgeeiENSG00000169689.
CleanExiHS_STRA13.
ExpressionAtlasiA8MT69. baseline and differential.
GenevisibleiA8MT69. HS.

Interactioni

Subunit structurei

Heterodimer with CENPX, sometimes called MHF; this interaction stabilizes both partners (PubMed:19620631, PubMed:20347428, PubMed:20347429, PubMed:24522885). MHF heterodimers can assemble to form tetrameric structures (PubMed:22304917). MHF also coassemble with CENPT-CENPW heterodimers at centromeres to form the tetrameric CENP-T-W-S-X complex (PubMed:22304917, PubMed:24522885). Forms a discrete complex with FANCM and CENPX, called FANCM-MHF; this interaction, probably mediated by direct binding between CENPS and FANCM, leads to synergistic activation of double-stranded DNA binding and strongly stimulates FANCM-mediated DNA remodeling (PubMed:20347428, PubMed:20347429). Recruited by FANCM to the Fanconi anemia (FA) core complex, which consists of CENPS, CENPX, FANCA, FANCB, FANCC, FANCE, FANCF, FANCG, FANCL, FANCM, FAAP24 and FAAP100. The FA core complex associates with Bloom syndrome (BLM) complex, which consists of at least BLM, DNA topoisomerase 3-alpha (TOP3A), RMI1/BLAP75, RPA1/RPA70 and RPA2/RPA32. The super complex between FA and BLM is called BRAFT (PubMed:20347428, PubMed:20347429).5 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi128376. 24 interactors.
IntActiA8MT69. 9 interactors.

Structurei

Secondary structure

181
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi12 – 20Combined sources9
Helixi32 – 59Combined sources28
Beta strandi63 – 65Combined sources3
Helixi67 – 80Combined sources14

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4DRAX-ray2.41E/F/G/H1-81[»]
4DRBX-ray2.63J/K/L/M/N/O1-81[»]
4E44X-ray2.10B/D1-81[»]
4E45X-ray2.00B/D/G/I/L/N1-81[»]
4NDYX-ray7.00B/D/H/L/M/N/U/V/W/X8-81[»]
4NE1X-ray6.50B/D/H/L/M/N/U/V/W/X/Z/b/d/h/i/j/o/p/q/r8-81[»]
4NE3X-ray1.80B8-81[»]
4NE5X-ray2.50B/D/F/H8-81[»]
4NE6X-ray2.10B/D8-81[»]
ProteinModelPortaliA8MT69.
SMRiA8MT69.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the CENPX family.Curated

Phylogenomic databases

GeneTreeiENSGT00390000002725.
HOGENOMiHOG000231242.
InParanoidiA8MT69.
KOiK15360.
PhylomeDBiA8MT69.

Family and domain databases

InterProiView protein in InterPro
IPR018552. CENP-X.
PfamiView protein in Pfam
PF09415. CENP-X. 1 hit.

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: A8MT69-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MEGAGAGSGF RKELVSRLLH LHFKDDKTKV SGDALQLMVE LLKVFVVEAA
60 70 80
VRGVRQAQAE DALRVDVDQL EKVLPQLLLD F
Length:81
Mass (Da):8,959
Last modified:December 4, 2007 - v1
Checksum:i0BA47A4F0FFD2978
GO
Isoform 2 (identifier: A8MT69-2) [UniParc]FASTAAdd to basket
Also known as: Variant A

The sequence of this isoform differs from the canonical sequence as follows:
     30-47: Missing.

Show »
Length:63
Mass (Da):7,017
Checksum:iB751CBF3D27E6B1A
GO
Isoform 3 (identifier: A8MT69-3) [UniParc]FASTAAdd to basket
Also known as: Variant B

The sequence of this isoform differs from the canonical sequence as follows:
     30-47: Missing.
     73-77: Missing.

Show »
Length:58
Mass (Da):6,466
Checksum:i5166DA91818795E5
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti65V → A in AAH09571 (PubMed:15489334).Curated1
Sequence conflicti73V → L in AAB53638 (Ref. 1) Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_03394830 – 47Missing in isoform 2 and isoform 3. 2 PublicationsAdd BLAST18
Alternative sequenceiVSP_03394973 – 77Missing in isoform 3. 2 Publications5

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U95006 mRNA. Translation: AAB53638.1.
U95007 mRNA. Translation: AAB53639.1.
AC137723 Genomic DNA. No translation available.
BC009571 mRNA. Translation: AAH09571.1.
BC011610 mRNA. Translation: AAH11610.1.
CCDSiCCDS32772.1. [A8MT69-2]
CCDS59302.1. [A8MT69-3]
CCDS59303.1. [A8MT69-1]
RefSeqiNP_001257935.1. NM_001271006.1. [A8MT69-1]
NP_001257936.1. NM_001271007.1. [A8MT69-3]
NP_001317465.1. NM_001330536.1.
NP_659435.2. NM_144998.3. [A8MT69-2]
UniGeneiHs.37616.

Genome annotation databases

EnsembliENST00000306704; ENSP00000302951; ENSG00000169689. [A8MT69-2]
ENST00000392359; ENSP00000376168; ENSG00000169689. [A8MT69-1]
ENST00000580435; ENSP00000462015; ENSG00000169689. [A8MT69-3]
GeneIDi201254.
KEGGihsa:201254.
UCSCiuc002kdc.5. human. [A8MT69-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U95006 mRNA. Translation: AAB53638.1.
U95007 mRNA. Translation: AAB53639.1.
AC137723 Genomic DNA. No translation available.
BC009571 mRNA. Translation: AAH09571.1.
BC011610 mRNA. Translation: AAH11610.1.
CCDSiCCDS32772.1. [A8MT69-2]
CCDS59302.1. [A8MT69-3]
CCDS59303.1. [A8MT69-1]
RefSeqiNP_001257935.1. NM_001271006.1. [A8MT69-1]
NP_001257936.1. NM_001271007.1. [A8MT69-3]
NP_001317465.1. NM_001330536.1.
NP_659435.2. NM_144998.3. [A8MT69-2]
UniGeneiHs.37616.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4DRAX-ray2.41E/F/G/H1-81[»]
4DRBX-ray2.63J/K/L/M/N/O1-81[»]
4E44X-ray2.10B/D1-81[»]
4E45X-ray2.00B/D/G/I/L/N1-81[»]
4NDYX-ray7.00B/D/H/L/M/N/U/V/W/X8-81[»]
4NE1X-ray6.50B/D/H/L/M/N/U/V/W/X/Z/b/d/h/i/j/o/p/q/r8-81[»]
4NE3X-ray1.80B8-81[»]
4NE5X-ray2.50B/D/F/H8-81[»]
4NE6X-ray2.10B/D8-81[»]
ProteinModelPortaliA8MT69.
SMRiA8MT69.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi128376. 24 interactors.
IntActiA8MT69. 9 interactors.

PTM databases

iPTMnetiA8MT69.
PhosphoSitePlusiA8MT69.

Proteomic databases

EPDiA8MT69.
MaxQBiA8MT69.
PRIDEiA8MT69.
TopDownProteomicsiA8MT69-1. [A8MT69-1]
A8MT69-2. [A8MT69-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000306704; ENSP00000302951; ENSG00000169689. [A8MT69-2]
ENST00000392359; ENSP00000376168; ENSG00000169689. [A8MT69-1]
ENST00000580435; ENSP00000462015; ENSG00000169689. [A8MT69-3]
GeneIDi201254.
KEGGihsa:201254.
UCSCiuc002kdc.5. human. [A8MT69-1]

Organism-specific databases

CTDi201254.
DisGeNETi201254.
GeneCardsiMHF2.
H-InvDBHIX0014271.
HGNCiHGNC:11422. CENPX.
MIMi615128. gene.
neXtProtiNX_A8MT69.
OpenTargetsiENSG00000169689.
PharmGKBiPA36223.
GenAtlasiSearch...

Phylogenomic databases

GeneTreeiENSGT00390000002725.
HOGENOMiHOG000231242.
InParanoidiA8MT69.
KOiK15360.
PhylomeDBiA8MT69.

Enzyme and pathway databases

ReactomeiR-HSA-606279. Deposition of new CENPA-containing nucleosomes at the centromere.
R-HSA-6783310. Fanconi Anemia Pathway.
SIGNORiA8MT69.

Miscellaneous databases

GenomeRNAii201254.
PROiA8MT69.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000169689.
CleanExiHS_STRA13.
ExpressionAtlasiA8MT69. baseline and differential.
GenevisibleiA8MT69. HS.

Family and domain databases

InterProiView protein in InterPro
IPR018552. CENP-X.
PfamiView protein in Pfam
PF09415. CENP-X. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiCENPX_HUMAN
AccessioniPrimary (citable) accession number: A8MT69
Secondary accession number(s): O00281
, O00282, Q96DD4, Q96F51
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 20, 2008
Last sequence update: December 4, 2007
Last modified: March 15, 2017
This is version 86 of the entry and version 1 of the sequence. See 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 17
    Human chromosome 17: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. 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.