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Protein

Centromere protein J

Gene

CENPJ

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

Functioni

Plays an important role in cell division and centrosome function by participating in centriole duplication. Inhibits microtubule nucleation from the centrosome. Required for centriole elongation and for STIL-mediated centriole amplification (PubMed:22020124).4 Publications

GO - Molecular functioni

  • protein domain specific binding Source: UniProtKB
  • protein kinase binding Source: UniProtKB
  • tubulin binding Source: UniProtKB

GO - Biological processi

  • cell division Source: UniProtKB
  • centriole elongation Source: UniProtKB
  • centriole replication Source: UniProtKB
  • DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest Source: Reactome
  • G2/M transition of mitotic cell cycle Source: Reactome
  • microtubule nucleation Source: UniProtKB
  • microtubule polymerization Source: UniProtKB
  • motile cilium assembly Source: Ensembl
  • positive regulation of non-motile cilium assembly Source: Ensembl
  • regulation of centriole replication Source: UniProtKB
  • smoothened signaling pathway Source: Ensembl
Complete GO annotation...

Enzyme and pathway databases

BioCyciZFISH:ENSG00000151849-MONOMER.
ReactomeiR-HSA-2565942. Regulation of PLK1 Activity at G2/M Transition.
R-HSA-380259. Loss of Nlp from mitotic centrosomes.
R-HSA-380270. Recruitment of mitotic centrosome proteins and complexes.
R-HSA-5620912. Anchoring of the basal body to the plasma membrane.
R-HSA-6804115. TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain.
R-HSA-8854518. AURKA Activation by TPX2.
SIGNORiQ9HC77.

Names & Taxonomyi

Protein namesi
Recommended name:
Centromere protein J
Short name:
CENP-J
Alternative name(s):
Centrosomal P4.1-associated protein
LAG-3-associated protein
LYST-interacting protein 1
Gene namesi
Name:CENPJ
Synonyms:CPAP, LAP, LIP1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 13

Organism-specific databases

HGNCiHGNC:17272. CENPJ.

Subcellular locationi

GO - Cellular componenti

  • centriole Source: UniProtKB
  • centrosome Source: UniProtKB
  • ciliary basal body Source: Ensembl
  • cytosol Source: Reactome
  • gamma-tubulin small complex Source: UniProtKB
  • microtubule Source: UniProtKB-KW
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Cytoskeleton, Microtubule

Pathology & Biotechi

Involvement in diseasei

Microcephaly 6, primary, autosomal recessive (MCPH6)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disease defined as a head circumference more than 3 standard deviations below the age-related mean. Brain weight is markedly reduced and the cerebral cortex is disproportionately small. Despite this marked reduction in size, the gyral pattern is relatively well preserved, with no major abnormality in cortical architecture. Affected individuals are mentally retarded. Primary microcephaly is further defined by the absence of other syndromic features or significant neurological deficits due to degenerative brain disorder.
See also OMIM:608393
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0324331235E → V in MCPH6; reduced interaction with STIL. 2 PublicationsCorresponds to variant rs121434311dbSNPEnsembl.1
Seckel syndrome 4 (SCKL4)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA rare autosomal recessive disorder characterized by proportionate dwarfism of prenatal onset associated with low birth weight, growth retardation, severe microcephaly with a bird-headed like appearance, and mental retardation.
See also OMIM:613676

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi589S → A: Abolishes phosphorylation by PLK2 and procentriole formation; when associated with A-595. 1 Publication1
Mutagenesisi595S → A: Abolishes phosphorylation by PLK2 and procentriole formation; when associated with A-589. 1 Publication1

Keywords - Diseasei

Disease mutation, Dwarfism, Mental retardation, Primary microcephaly

Organism-specific databases

DisGeNETi55835.
MalaCardsiCENPJ.
MIMi608393. phenotype.
613676. phenotype.
OpenTargetsiENSG00000151849.
Orphaneti2512. Autosomal recessive primary microcephaly.
808. Seckel syndrome.
PharmGKBiPA26403.

Polymorphism and mutation databases

BioMutaiCENPJ.
DMDMi62899891.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000894801 – 1338Centromere protein JAdd BLAST1338

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei260PhosphoserineCombined sources1
Modified residuei316PhosphoserineCombined sources1
Modified residuei540PhosphoserineCombined sources1
Modified residuei589Phosphoserine; by PLK21 Publication1
Modified residuei595Phosphoserine; by PLK2 and PLK41 Publication1
Modified residuei759PhosphoserineCombined sources1

Post-translational modificationi

Phosphorylation at Ser-589 and Ser-595 by PLK2 is required for procentriole formation and centriole elongation. Phosphorylation by PLK2 oscillates during the cell cycle: it increases at G1/S transition and decreases during the exit from mitosis. Phosphorylation at Ser-595 is also mediated by PLK4 but is not a critical step in PLK4 function in procentriole assembly.1 Publication

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ9HC77.
MaxQBiQ9HC77.
PaxDbiQ9HC77.
PeptideAtlasiQ9HC77.
PRIDEiQ9HC77.

PTM databases

iPTMnetiQ9HC77.
PhosphoSitePlusiQ9HC77.

Expressioni

Gene expression databases

BgeeiENSG00000151849.
CleanExiHS_CENPJ.
ExpressionAtlasiQ9HC77. baseline and differential.
GenevisibleiQ9HC77. HS.

Interactioni

Subunit structurei

Part of a ternary complex composed of SASS6, CENPJ and CEP350. Associated with the gamma-tubulin complex. Interacts with the head domain of EPB41. Interacts with LYST. Interacts with CEP152 (via C-terminus). Interacts with STIL (PubMed:22020124, PubMed:25385835). Forms a complex with STIL and SASS6 (PubMed:22020124).6 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
CEP135Q66GS98EBI-946194,EBI-1046993
CICQ96RK02EBI-946194,EBI-945857
GFI1BQ5VTD92EBI-946194,EBI-946212
PLK2Q9NYY33EBI-946194,EBI-721354
STILQ1546813EBI-946194,EBI-7488405
TNKSO952714EBI-946194,EBI-1105254
YWHAGP619813EBI-946194,EBI-359832

GO - Molecular functioni

  • protein domain specific binding Source: UniProtKB
  • protein kinase binding Source: UniProtKB
  • tubulin binding Source: UniProtKB

Protein-protein interaction databases

BioGridi120939. 145 interactors.
DIPiDIP-49904N.
IntActiQ9HC77. 129 interactors.
STRINGi9606.ENSP00000371308.

Structurei

Secondary structure

11338
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi380 – 384Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5ITZX-ray2.20D311-422[»]
ProteinModelPortaliQ9HC77.
SMRiQ9HC77.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni895 – 1338Interaction with STIL2 PublicationsAdd BLAST444

Sequence similaritiesi

Belongs to the TCP10 family.Curated

Phylogenomic databases

eggNOGiENOG410IEFU. Eukaryota.
ENOG4110DC4. LUCA.
GeneTreeiENSGT00530000063927.
HOGENOMiHOG000111541.
HOVERGENiHBG050894.
InParanoidiQ9HC77.
KOiK11502.
OMAiDERTWTD.
OrthoDBiEOG091G00Z1.
PhylomeDBiQ9HC77.
TreeFamiTF343156.

Family and domain databases

InterProiIPR033068. CENP-J.
IPR009852. Tcp10_C_dom.
IPR026581. TCP10_fam.
[Graphical view]
PANTHERiPTHR10331. PTHR10331. 1 hit.
PTHR10331:SF23. PTHR10331:SF23. 1 hit.
PfamiPF07202. Tcp10_C. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9HC77-1) [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
MFLMPTSSEL NSGQNFLTQW MTNPSRAGVI LNRGFPILEA DKEKRAAVDI
60 70 80 90 100
STSFPIKGTH FSDSFSFINE EDSLLEEQKL ESNNPYKPQS DKSETHTAFP
110 120 130 140 150
CIKKGPQVAA CHSAPGHQEE NKNDFIPDLA SEFKEGAYKD PLFKKLEQLK
160 170 180 190 200
EVQQKKQEQL KRQQLEQLQR LMEEQEKLLT MVSGQCTLPG LSLLPDDQSQ
210 220 230 240 250
KHRSPGNTTT GERATCCFPS YVYPDPTQEE TYPSNILSHE QSNFCRTAHG
260 270 280 290 300
DFVLTSKRAS PNLFSEAQYQ EAPVEKNNLK EENRNHPTGE SILCWEKVTE
310 320 330 340 350
QIQEANDKNL QKHDDSSEVA NIEERPIKAA IGERKQTFED YLEEQIQLEE
360 370 380 390 400
QELKQKQLKE AEGPLPIKAK PKQPFLKRGE GLARFTNAKS KFQKGKESKL
410 420 430 440 450
VTNQSTSEDQ PLFKMDRQQL QRKTALKNKE LCADNPILKK DSKARTKSGS
460 470 480 490 500
VTLSQKPKML KCSNRKSLSP SGLKIQTGKK CDGQFRDQIK FENKVTSNNK
510 520 530 540 550
ENVTECPKPC DTGCTGWNKT QGKDRLPLST GPASRLAAKS PIRETMKESE
560 570 580 590 600
SSLDVSLQKK LETWEREKEK ENLELDEFLF LEQAADEISF SSNSSFVLKI
610 620 630 640 650
LERDQQICKG HRMSSTPVKA VPQKTNPADP ISHCNRSEDL DHTAREKESE
660 670 680 690 700
CEVAPKQLHS LSSADELREQ PCKIRKAVQK STSENQTEWN ARDDEGVPNS
710 720 730 740 750
DSSTDSEEQL DVTIKPSTED RERGISSRED SPQVCDDKGP FKDTRTQEDK
760 770 780 790 800
RRDVDLDLSD KDYSSDESIM ESIKHKVSEP SRSSSLSLSK MDFDDERTWT
810 820 830 840 850
DLEENLCNHD VVLGNESTYG TPQTCYPNNE IGILDKTIKR KIAPVKRGED
860 870 880 890 900
LSKSRRSRSP PTSELMMKFF PSLKPKPKSD SHLGNELKLN ISQDQPPGDN
910 920 930 940 950
ARSQVLREKI IELETEIEKF KAENASLAKL RIERESALEK LRKEIADFEQ
960 970 980 990 1000
QKAKELARIE EFKKEEMRKL QKERKVFEKY TTAARTFPDK KEREEIQTLK
1010 1020 1030 1040 1050
QQIADLREDL KRKETKWSST HSRLRSQIQM LVRENTDLRE EIKVMERFRL
1060 1070 1080 1090 1100
DAWKRAEAIE SSLEVEKKDK LANTSVRFQN SQISSGTQVE KYKKNYLPMQ
1110 1120 1130 1140 1150
GNPPRRSKSA PPRDLGNLDK GQAASPREPL EPLNFPDPEY KEEEEDQDIQ
1160 1170 1180 1190 1200
GEISHPDGKV EKVYKNGCRV ILFPNGTRKE VSADGKTITV TFFNGDVKQV
1210 1220 1230 1240 1250
MPDQRVIYYY AAAQTTHTTY PEGLEVLHFS SGQIEKHYPD GRKEITFPDQ
1260 1270 1280 1290 1300
TVKNLFPDGQ EESIFPDGTI VRVQRDGNKL IEFNNGQREL HTAQFKRREY
1310 1320 1330
PDGTVKTVYA NGHQETKYRS GRIRVKDKEG NVLMDTEL
Length:1,338
Mass (Da):153,000
Last modified:April 26, 2005 - v2
Checksum:iB4E6531B62A30F2D
GO
Isoform 2 (identifier: Q9HC77-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1073-1086: NTSVRFQNSQISSG → AIHLEDPSLHLLVI
     1087-1338: Missing.

Note: No experimental confirmation available.
Show »
Length:1,086
Mass (Da):124,244
Checksum:i4AD0A29BE7DD1523
GO

Sequence cautioni

The sequence AAH24209 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti129L → R in AAG21074 (PubMed:11003675).Curated1
Sequence conflicti1142E → R in AAG49440 (PubMed:11984006).Curated1
Sequence conflicti1224L → W in AAG49440 (PubMed:11984006).Curated1
Sequence conflicti1333L → S in AAG21074 (PubMed:11003675).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03242721M → V.Corresponds to variant rs35498994dbSNPEnsembl.1
Natural variantiVAR_03242855P → A.Corresponds to variant rs17081389dbSNPEnsembl.1
Natural variantiVAR_03242963D → H.Corresponds to variant rs7336216dbSNPEnsembl.1
Natural variantiVAR_03243085P → T.Corresponds to variant rs9511510dbSNPEnsembl.1
Natural variantiVAR_032431151E → G.Corresponds to variant rs34177811dbSNPEnsembl.1
Natural variantiVAR_032432879S → A.Corresponds to variant rs17402892dbSNPEnsembl.1
Natural variantiVAR_0324331235E → V in MCPH6; reduced interaction with STIL. 2 PublicationsCorresponds to variant rs121434311dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0568311073 – 1086NTSVR…QISSG → AIHLEDPSLHLLVI in isoform 2. 1 PublicationAdd BLAST14
Alternative sequenceiVSP_0568321087 – 1338Missing in isoform 2. 1 PublicationAdd BLAST252

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF139625 mRNA. Translation: AAG21074.1.
AL833182 mRNA. Translation: CAI46195.1.
AL354798 Genomic DNA. Translation: CAI16635.1.
CH471075 Genomic DNA. Translation: EAX08354.1.
BC024209 mRNA. Translation: AAH24209.3. Different initiation.
BC113110 mRNA. Translation: AAI13111.1.
BC113662 mRNA. Translation: AAI13663.1.
BC113664 mRNA. Translation: AAI13665.1.
AJ303006 mRNA. Translation: CAC80028.1.
AF141337 mRNA. Translation: AAG49440.1.
CCDSiCCDS9310.1. [Q9HC77-1]
RefSeqiNP_060921.3. NM_018451.4. [Q9HC77-1]
UniGeneiHs.513379.
Hs.741581.

Genome annotation databases

EnsembliENST00000381884; ENSP00000371308; ENSG00000151849. [Q9HC77-1]
ENST00000616936; ENSP00000477511; ENSG00000151849. [Q9HC77-2]
GeneIDi55835.
KEGGihsa:55835.
UCSCiuc001upt.6. human. [Q9HC77-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF139625 mRNA. Translation: AAG21074.1.
AL833182 mRNA. Translation: CAI46195.1.
AL354798 Genomic DNA. Translation: CAI16635.1.
CH471075 Genomic DNA. Translation: EAX08354.1.
BC024209 mRNA. Translation: AAH24209.3. Different initiation.
BC113110 mRNA. Translation: AAI13111.1.
BC113662 mRNA. Translation: AAI13663.1.
BC113664 mRNA. Translation: AAI13665.1.
AJ303006 mRNA. Translation: CAC80028.1.
AF141337 mRNA. Translation: AAG49440.1.
CCDSiCCDS9310.1. [Q9HC77-1]
RefSeqiNP_060921.3. NM_018451.4. [Q9HC77-1]
UniGeneiHs.513379.
Hs.741581.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5ITZX-ray2.20D311-422[»]
ProteinModelPortaliQ9HC77.
SMRiQ9HC77.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi120939. 145 interactors.
DIPiDIP-49904N.
IntActiQ9HC77. 129 interactors.
STRINGi9606.ENSP00000371308.

PTM databases

iPTMnetiQ9HC77.
PhosphoSitePlusiQ9HC77.

Polymorphism and mutation databases

BioMutaiCENPJ.
DMDMi62899891.

Proteomic databases

EPDiQ9HC77.
MaxQBiQ9HC77.
PaxDbiQ9HC77.
PeptideAtlasiQ9HC77.
PRIDEiQ9HC77.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000381884; ENSP00000371308; ENSG00000151849. [Q9HC77-1]
ENST00000616936; ENSP00000477511; ENSG00000151849. [Q9HC77-2]
GeneIDi55835.
KEGGihsa:55835.
UCSCiuc001upt.6. human. [Q9HC77-1]

Organism-specific databases

CTDi55835.
DisGeNETi55835.
GeneCardsiCENPJ.
GeneReviewsiCENPJ.
HGNCiHGNC:17272. CENPJ.
MalaCardsiCENPJ.
MIMi608393. phenotype.
609279. gene.
613676. phenotype.
neXtProtiNX_Q9HC77.
OpenTargetsiENSG00000151849.
Orphaneti2512. Autosomal recessive primary microcephaly.
808. Seckel syndrome.
PharmGKBiPA26403.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IEFU. Eukaryota.
ENOG4110DC4. LUCA.
GeneTreeiENSGT00530000063927.
HOGENOMiHOG000111541.
HOVERGENiHBG050894.
InParanoidiQ9HC77.
KOiK11502.
OMAiDERTWTD.
OrthoDBiEOG091G00Z1.
PhylomeDBiQ9HC77.
TreeFamiTF343156.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000151849-MONOMER.
ReactomeiR-HSA-2565942. Regulation of PLK1 Activity at G2/M Transition.
R-HSA-380259. Loss of Nlp from mitotic centrosomes.
R-HSA-380270. Recruitment of mitotic centrosome proteins and complexes.
R-HSA-5620912. Anchoring of the basal body to the plasma membrane.
R-HSA-6804115. TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain.
R-HSA-8854518. AURKA Activation by TPX2.
SIGNORiQ9HC77.

Miscellaneous databases

GeneWikiiCENPJ.
GenomeRNAii55835.
PROiQ9HC77.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000151849.
CleanExiHS_CENPJ.
ExpressionAtlasiQ9HC77. baseline and differential.
GenevisibleiQ9HC77. HS.

Family and domain databases

InterProiIPR033068. CENP-J.
IPR009852. Tcp10_C_dom.
IPR026581. TCP10_fam.
[Graphical view]
PANTHERiPTHR10331. PTHR10331. 1 hit.
PTHR10331:SF23. PTHR10331:SF23. 1 hit.
PfamiPF07202. Tcp10_C. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCENPJ_HUMAN
AccessioniPrimary (citable) accession number: Q9HC77
Secondary accession number(s): Q2KHM6
, Q5JPD5, Q5T6R5, Q96KS5, Q9C067
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 26, 2005
Last sequence update: April 26, 2005
Last modified: November 30, 2016
This is version 131 of the entry and version 2 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 13
    Human chromosome 13: 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. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. 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.