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Protein

Kelch-like protein 22

Gene

KLHL22

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

Functioni

Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex required for chromosome alignment and localization of PLK1 at kinetochores. The BCR(KLHL22) ubiquitin ligase complex mediates monoubiquitination of PLK1, leading to PLK1 dissociation from phosphoreceptor proteins and subsequent removal from kinetochores, allowing silencing of the spindle assembly checkpoint (SAC) and chromosome segregation. Monoubiquitination of PLK1 does not lead to PLK1 degradation.2 Publications

Pathwayi: protein ubiquitination

This protein is involved in the pathway protein ubiquitination, which is part of Protein modification.
View all proteins of this organism that are known to be involved in the pathway protein ubiquitination and in Protein modification.

GO - Biological processi

  • cell division Source: UniProtKB
  • mitotic sister chromatid segregation Source: UniProtKB
  • mitotic spindle assembly checkpoint Source: UniProtKB
  • post-translational protein modification Source: Reactome
  • protein monoubiquitination Source: UniProtKB

Keywordsi

Biological processCell cycle, Cell division, Mitosis, Ubl conjugation pathway

Enzyme and pathway databases

ReactomeiR-HSA-8951664 Neddylation
R-HSA-983168 Antigen processing: Ubiquitination & Proteasome degradation
UniPathwayiUPA00143

Names & Taxonomyi

Protein namesi
Recommended name:
Kelch-like protein 22
Gene namesi
Name:KLHL22
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 22

Organism-specific databases

EuPathDBiHostDB:ENSG00000099910.16
HGNCiHGNC:25888 KLHL22
neXtProtiNX_Q53GT1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Cytoskeleton

Pathology & Biotechi

Involvement in diseasei

Defects in KLHL22 has been found in a patient with isolated coloboma, a defect of the eye characterized by the absence of ocular structures due to abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure). Isolated colobomas may be associated with an abnormally small eye (microphthalmia) or small cornea.1 Publication

Keywords - Diseasei

Disease mutation

Organism-specific databases

OpenTargetsiENSG00000099910
PharmGKBiPA142671574

Polymorphism and mutation databases

BioMutaiKLHL22
DMDMi109892504

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00002421552 – 634Kelch-like protein 22Add BLAST633

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1
Modified residuei463PhosphothreonineBy similarity1
Modified residuei466PhosphotyrosineBy similarity1
Modified residuei475PhosphothreonineBy similarity1
Modified residuei605PhosphothreonineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ53GT1
MaxQBiQ53GT1
PaxDbiQ53GT1
PeptideAtlasiQ53GT1
PRIDEiQ53GT1

PTM databases

iPTMnetiQ53GT1
PhosphoSitePlusiQ53GT1

Expressioni

Gene expression databases

BgeeiENSG00000099910
CleanExiHS_KLHL22
ExpressionAtlasiQ53GT1 baseline and differential
GenevisibleiQ53GT1 HS

Interactioni

Subunit structurei

Component of the BCR(KLHL22) E3 ubiquitin ligase complex, at least composed of CUL3, KLHL22 and RBX1. Interacts with PLK1.2 Publications

Protein-protein interaction databases

BioGridi124308, 51 interactors
IntActiQ53GT1, 9 interactors
STRINGi9606.ENSP00000331682

Structurei

3D structure databases

ProteinModelPortaliQ53GT1
SMRiQ53GT1
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini50 – 117BTBPROSITE-ProRule annotationAdd BLAST68
Repeati299 – 349Kelch 1Add BLAST51
Repeati350 – 399Kelch 2Add BLAST50
Repeati400 – 446Kelch 3Add BLAST47
Repeati448 – 493Kelch 4Add BLAST46
Repeati494 – 544Kelch 5Add BLAST51
Repeati545 – 593Kelch 6Add BLAST49

Keywords - Domaini

Kelch repeat, Repeat

Phylogenomic databases

eggNOGiKOG4441 Eukaryota
ENOG410XNX8 LUCA
GeneTreeiENSGT00760000118825
HOGENOMiHOG000231583
HOVERGENiHBG105890
InParanoidiQ53GT1
KOiK10459
OMAiQGPQTEL
OrthoDBiEOG091G02OF
PhylomeDBiQ53GT1
TreeFamiTF328485

Family and domain databases

Gene3Di2.120.10.80, 1 hit
InterProiView protein in InterPro
IPR011705 BACK
IPR017096 BTB-kelch_protein
IPR000210 BTB/POZ_dom
IPR015915 Kelch-typ_b-propeller
IPR006652 Kelch_1
IPR030575 KLHL22
IPR011333 SKP1/BTB/POZ_sf
PANTHERiPTHR24412:SF193 PTHR24412:SF193, 1 hit
PfamiView protein in Pfam
PF07707 BACK, 1 hit
PF00651 BTB, 1 hit
PF01344 Kelch_1, 2 hits
PIRSFiPIRSF037037 Kelch-like_protein_gigaxonin, 1 hit
SMARTiView protein in SMART
SM00875 BACK, 1 hit
SM00225 BTB, 1 hit
SM00612 Kelch, 6 hits
SUPFAMiSSF117281 SSF117281, 1 hit
SSF54695 SSF54695, 1 hit
PROSITEiView protein in PROSITE
PS50097 BTB, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

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

Isoform 1 (identifier: Q53GT1-1) [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
MAEEQEFTQL CKLPAQPSHP HCVNNTYRSA QHSQALLRGL LALRDSGILF
60 70 80 90 100
DVVLVVEGRH IEAHRILLAA SCDYFRGMFA GGLKEMEQEE VLIHGVSYNA
110 120 130 140 150
MCQILHFIYT SELELSLSNV QETLVAACQL QIPEIIHFCC DFLMSWVDEE
160 170 180 190 200
NILDVYRLAE LFDLSRLTEQ LDTYILKNFV AFSRTDKYRQ LPLEKVYSLL
210 220 230 240 250
SSNRLEVSCE TEVYEGALLY HYSLEQVQAD QISLHEPPKL LETVRFPLME
260 270 280 290 300
AEVLQRLHDK LDPSPLRDTV ASALMYHRNE SLQPSLQSPQ TELRSDFQCV
310 320 330 340 350
VGFGGIHSTP STVLSDQAKY LNPLLGEWKH FTASLAPRMS NQGIAVLNNF
360 370 380 390 400
VYLIGGDNNV QGFRAESRCW RYDPRHNRWF QIQSLQQEHA DLSVCVVGRY
410 420 430 440 450
IYAVAGRDYH NDLNAVERYD PATNSWAYVA PLKREVYAHA GATLEGKMYI
460 470 480 490 500
TCGRRGEDYL KETHCYDPGS NTWHTLADGP VRRAWHGMAT LLNKLYVIGG
510 520 530 540 550
SNNDAGYRRD VHQVACYSCT SGQWSSVCPL PAGHGEPGIA VLDNRIYVLG
560 570 580 590 600
GRSHNRGSRT GYVHIYDVEK DCWEEGPQLD NSISGLAACV LTLPRSLLLE
610 620 630
PPRGTPDRSQ ADPDFASEVM SVSDWEEFDN SSED
Length:634
Mass (Da):71,667
Last modified:June 27, 2006 - v2
Checksum:i63A7AF7AAE0F56D7
GO
Isoform 2 (identifier: Q53GT1-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-143: Missing.

Note: No experimental confirmation available.
Show »
Length:491
Mass (Da):55,567
Checksum:iF79566910B725C58
GO

Sequence cautioni

The sequence BAB55007 differs from that shown. Aberrant splicing.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti352Y → H in BAD96570 (Ref. 3) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07985420P → Q Found in a patient with isolated coloboma; unknown pathological significance. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0570281 – 143Missing in isoform 2. 1 PublicationAdd BLAST143

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR456352 mRNA Translation: CAG30238.1
AK027266 mRNA Translation: BAB55007.1 Sequence problems.
AK290669 mRNA Translation: BAF83358.1
AK294682 mRNA Translation: BAH11847.1
AK222850 mRNA Translation: BAD96570.1
AC007731 Genomic DNA No translation available.
CH471176 Genomic DNA Translation: EAX02961.1
CH471176 Genomic DNA Translation: EAX02962.1
CH471176 Genomic DNA Translation: EAX02963.1
BC015923 mRNA Translation: AAH15923.1
CCDSiCCDS13780.1 [Q53GT1-1]
RefSeqiNP_116164.2, NM_032775.3 [Q53GT1-1]
XP_016884510.1, XM_017029021.1 [Q53GT1-1]
UniGeneiHs.517419

Genome annotation databases

EnsembliENST00000328879; ENSP00000331682; ENSG00000099910 [Q53GT1-1]
GeneIDi84861
KEGGihsa:84861
UCSCiuc002zsl.2 human [Q53GT1-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiKLH22_HUMAN
AccessioniPrimary (citable) accession number: Q53GT1
Secondary accession number(s): A8K3Q4
, A8MTV3, B7Z2G1, D3DX30, Q96B68, Q96KC6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 27, 2006
Last sequence update: June 27, 2006
Last modified: April 25, 2018
This is version 117 of the entry and version 2 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

Complete proteome, Reference proteome

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