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Protein

WD repeat-containing protein 26

Gene

WDR26

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

Functioni

G-beta-like protein involved in cell signal transduction (PubMed:15378603, PubMed:19446606, PubMed:22065575, PubMed:23625927, PubMed:27098453, PubMed:26895380). Acts as a negative regulator in MAPK signaling pathway (PubMed:15378603). Functions as a scaffolding protein to promote G beta:gamma-mediated PLCB2 plasma membrane translocation and subsequent activation in leukocytes (PubMed:22065575, PubMed:23625927). Core component of the CTLH E3 ubiquitin-protein ligase complex that selectively accepts ubiquitin from UBE2H and mediates ubiquitination and subsequent proteasomal degradation of the transcription factor HBP1 (PubMed:29911972). Acts as a negative regulator of the canonical Wnt signaling pathway through preventing ubiquitination of beta-catenin CTNNB1 by the beta-catenin destruction complex, thus negatively regulating CTNNB1 degradation (PubMed:27098453). Serves as a scaffold to coordinate PI3K/AKT pathway-driven cell growth and migration (PubMed:26895380). Protects cells from oxidative stress-induced apoptosis via the down-regulation of AP-1 transcriptional activity as well as by inhibiting cytochrome c release from mitochondria (PubMed:19446606). Protects also cells by promoting hypoxia-mediated autophagy and mitophagy (By similarity).By similarity6 Publications

Enzyme and pathway databases

SignaLinkiQ9H7D7

Names & Taxonomyi

Protein namesi
Recommended name:
WD repeat-containing protein 26
Alternative name(s):
CUL4- and DDB1-associated WDR protein 2
Myocardial ischemic preconditioning up-regulated protein 2
Gene namesi
Name:WDR26
Synonyms:CDW2, MIP2
ORF Names:PRO0852
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000162923.14
HGNCiHGNC:21208 WDR26
MIMi617424 gene
neXtProtiNX_Q9H7D7

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, Mitochondrion, Nucleus

Pathology & Biotechi

Involvement in diseasei

Skraban-Deardorff syndrome (SKDEAS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal dominant syndrome characterized by psychomotor developmental delay, intellectual disability with delayed speech, febrile and non-febrile seizures, abnormal gait, and facial dysmorphism. Facial features include a prominent maxilla and upper lip that readily reveal the upper gingiva, widely spaced teeth, and a broad nasal tip.
See also OMIM:617616
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07929746 – 661Missing in SKDEAS. 1 PublicationAdd BLAST616
Natural variantiVAR_079298172W → R in SKDEAS; unknown pathological significance. 1 Publication1
Natural variantiVAR_079299215L → P in SKDEAS; unknown pathological significance. 1 Publication1
Natural variantiVAR_079300254S → R in SKDEAS; unknown pathological significance. 1 Publication1
Natural variantiVAR_079301279 – 661Missing in SKDEAS; unknown pathological significance. 1 PublicationAdd BLAST383
Natural variantiVAR_079302284D → N in SKDEAS; unknown pathological significance; slightly decreased protein expression;. 1 Publication1
Natural variantiVAR_079303426 – 661Missing in SKDEAS. 1 PublicationAdd BLAST236
Natural variantiVAR_079304428 – 661Missing in SKDEAS. 1 PublicationAdd BLAST234
Natural variantiVAR_079305524 – 661Missing in SKDEAS. 1 PublicationAdd BLAST138

Keywords - Diseasei

Disease mutation, Mental retardation

Organism-specific databases

DisGeNETi80232
MalaCardsiWDR26
MIMi617616 phenotype
OpenTargetsiENSG00000162923
Orphaneti513456 Intellectual disability-seizures-abnormal gait-facial dysmorphism syndrome
PharmGKBiPA134907873

Polymorphism and mutation databases

BioMutaiWDR26
DMDMi134047967

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000513731 – 661WD repeat-containing protein 26Add BLAST661

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei121PhosphoserineCombined sources1
Modified residuei123PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ9H7D7
MaxQBiQ9H7D7
PaxDbiQ9H7D7
PeptideAtlasiQ9H7D7
PRIDEiQ9H7D7
ProteomicsDBi81109
81110 [Q9H7D7-2]
81111 [Q9H7D7-3]
81112 [Q9H7D7-4]

PTM databases

iPTMnetiQ9H7D7
PhosphoSitePlusiQ9H7D7

Expressioni

Tissue specificityi

Broadly expressed, with highest levels in heart and skeletal muscle.1 Publication

Inductioni

Expression is significantly up-regulated by oxidative stress (PubMed:19446606).1 Publication

Gene expression databases

BgeeiENSG00000162923 Expressed in 225 organ(s), highest expression level in sperm
CleanExiHS_WDR26
ExpressionAtlasiQ9H7D7 baseline and differential
GenevisibleiQ9H7D7 HS

Organism-specific databases

HPAiHPA028539
HPA061094

Interactioni

Subunit structurei

Forms homooligomers (PubMed:23625927). Identified in the CTLH complex that contains GID4, RANBP9 and/or RANBP10, MKLN1, MAEA, RMND5A (or alternatively its paralog RMND5B), GID8, ARMC8, WDR26 and YPEL5 (PubMed:29911972). Within this complex, MAEA, RMND5A (or alternatively its paralog RMND5B), GID8, WDR26, and RANBP9 and/or RANBP10 form the catalytic core, while GID4, MKLN1, ARMC8 and YPEL5 have ancillary roles (PubMed:29911972). Interacts with DDB1-CUL4A/B E3 ligase complexes (PubMed:17041588). Forms a complex composed of at least WDR26, a G-beta:gamma unit, and PLCB2 (PubMed:22065575, PubMed:23625927). Interacts with AXIN1 (PubMed:27098453).5 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
AXIN1O151694EBI-1046864,EBI-710484

Protein-protein interaction databases

BioGridi123195, 94 interactors
CORUMiQ9H7D7
IntActiQ9H7D7, 38 interactors
MINTiQ9H7D7
STRINGi9606.ENSP00000408108

Structurei

3D structure databases

ProteinModelPortaliQ9H7D7
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini123 – 155LisHPROSITE-ProRule annotationAdd BLAST33
Domaini156 – 231CTLHPROSITE-ProRule annotationAdd BLAST76
Repeati353 – 392WD 1Add BLAST40
Repeati399 – 438WD 2Add BLAST40
Repeati444 – 484WD 3Add BLAST41
Repeati524 – 563WD 4Add BLAST40
Repeati566 – 608WD 5Add BLAST43
Repeati611 – 651WD 6Add BLAST41

Keywords - Domaini

Repeat, WD repeat

Phylogenomic databases

eggNOGiKOG0293 Eukaryota
ENOG410XQ2U LUCA
GeneTreeiENSGT00730000111021
HOVERGENiHBG083958
InParanoidiQ9H7D7
KOiK22382
OMAiQCLYHNT
OrthoDBiEOG091G05NI
PhylomeDBiQ9H7D7
TreeFamiTF314869

Family and domain databases

Gene3Di2.130.10.10, 1 hit
InterProiView protein in InterPro
IPR006595 CTLH_C
IPR006594 LisH
IPR015943 WD40/YVTN_repeat-like_dom_sf
IPR001680 WD40_repeat
IPR017986 WD40_repeat_dom
IPR036322 WD40_repeat_dom_sf
PfamiView protein in Pfam
PF00400 WD40, 4 hits
SMARTiView protein in SMART
SM00668 CTLH, 1 hit
SM00320 WD40, 5 hits
SUPFAMiSSF50978 SSF50978, 1 hit
PROSITEiView protein in PROSITE
PS50897 CTLH, 1 hit
PS50896 LISH, 1 hit
PS50082 WD_REPEATS_2, 3 hits
PS50294 WD_REPEATS_REGION, 1 hit

Sequences (4+)i

Sequence statusi: Complete.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 4 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9H7D7-1) [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
MQANGAGGGG GGGGGGGGGG GGGGGQGQTP ELACLSAQNG ESSPSSSSSA
60 70 80 90 100
GDLAHANGLL PSAPSAASNN SNSLNVNNGV PGGAAAASSA TVAAASATTA
110 120 130 140 150
ASSSLATPEL GSSLKKKKRL SQSDEDVIRL IGQHLNGLGL NQTVDLLMQE
160 170 180 190 200
SGCRLEHPSA TKFRNHVMEG DWDKAENDLN ELKPLVHSPH AIVVRGALEI
210 220 230 240 250
SQTLLGIIVR MKFLLLQQKY LEYLEDGKVL EALQVLRCEL TPLKYNTERI
260 270 280 290 300
HVLSGYLMCS HAEDLRAKAE WEGKGTASRS KLLDKLQTYL PPSVMLPPRR
310 320 330 340 350
LQTLLRQAVE LQRDRCLYHN TKLDNNLDSV SLLIDHVCSR RQFPCYTQQI
360 370 380 390 400
LTEHCNEVWF CKFSNDGTKL ATGSKDTTVI IWQVDPDTHL LKLLKTLEGH
410 420 430 440 450
AYGVSYIAWS PDDNYLVACG PDDCSELWLW NVQTGELRTK MSQSHEDSLT
460 470 480 490 500
SVAWNPDGKR FVTGGQRGQF YQCDLDGNLL DSWEGVRVQC LWCLSDGKTV
510 520 530 540 550
LASDTHQRIR GYNFEDLTDR NIVQEDHPIM SFTISKNGRL ALLNVATQGV
560 570 580 590 600
HLWDLQDRVL VRKYQGVTQG FYTIHSCFGG HNEDFIASGS EDHKVYIWHK
610 620 630 640 650
RSELPIAELT GHTRTVNCVS WNPQIPSMMA SASDDGTVRI WGPAPFIDHQ
660
NIEEECSSMD S
Length:661
Mass (Da):72,124
Last modified:March 20, 2007 - v3
Checksum:iE0B8AAFF44CB7DF7
GO
Isoform 2 (identifier: Q9H7D7-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     192-207: Missing.

Note: No experimental confirmation available.
Show »
Length:645
Mass (Da):70,460
Checksum:i664C8EA291526315
GO
Isoform 3 (identifier: Q9H7D7-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     194-216: VRGALEISQTLLGIIVRMKFLLL → AQTFSETSINFFPLTAAFCHVRG
     217-661: Missing.

Note: No experimental confirmation available.
Show »
Length:216
Mass (Da):21,314
Checksum:i0CA772E9A8A59012
GO
Isoform 4 (identifier: Q9H7D7-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     210-223: RMKFLLLQQKYLEY → VNTLLFLVSHLCLF
     224-661: Missing.

Note: No experimental confirmation available.
Show »
Length:223
Mass (Da):22,052
Checksum:i7880CBDF91D06DA0
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0Y9R3H0Y9R3_HUMAN
WD repeat-containing protein 26
WDR26
295Annotation score:
C9JCS7C9JCS7_HUMAN
WD repeat-containing protein 26
WDR26
125Annotation score:
H0Y917H0Y917_HUMAN
WD repeat-containing protein 26
WDR26
208Annotation score:

Sequence cautioni

The sequence AAG35477 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAH52301 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAH63817 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAO67709 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAQ74770 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence ABK41102 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence BAB14955 differs from that shown. Reason: Erroneous termination at position 274. Translated as Lys.Curated
The sequence BAD93124 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti8G → A in AAH34498 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07929746 – 661Missing in SKDEAS. 1 PublicationAdd BLAST616
Natural variantiVAR_079298172W → R in SKDEAS; unknown pathological significance. 1 Publication1
Natural variantiVAR_079299215L → P in SKDEAS; unknown pathological significance. 1 Publication1
Natural variantiVAR_079300254S → R in SKDEAS; unknown pathological significance. 1 Publication1
Natural variantiVAR_079301279 – 661Missing in SKDEAS; unknown pathological significance. 1 PublicationAdd BLAST383
Natural variantiVAR_079302284D → N in SKDEAS; unknown pathological significance; slightly decreased protein expression;. 1 Publication1
Natural variantiVAR_079303426 – 661Missing in SKDEAS. 1 PublicationAdd BLAST236
Natural variantiVAR_079304428 – 661Missing in SKDEAS. 1 PublicationAdd BLAST234
Natural variantiVAR_079305524 – 661Missing in SKDEAS. 1 PublicationAdd BLAST138

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_023895192 – 207Missing in isoform 2. 1 PublicationAdd BLAST16
Alternative sequenceiVSP_023896194 – 216VRGAL…KFLLL → AQTFSETSINFFPLTAAFCH VRG in isoform 3. 1 PublicationAdd BLAST23
Alternative sequenceiVSP_023897210 – 223RMKFL…KYLEY → VNTLLFLVSHLCLF in isoform 4. 1 PublicationAdd BLAST14
Alternative sequenceiVSP_023898217 – 661Missing in isoform 3. 1 PublicationAdd BLAST445
Alternative sequenceiVSP_023899224 – 661Missing in isoform 4. 1 PublicationAdd BLAST438

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB209887 mRNA Translation: BAD93124.1 Different initiation.
AC099790 Genomic DNA No translation available.
BC031471 mRNA Translation: AAH31471.2
BC034498 mRNA Translation: AAH34498.1
BC052301 mRNA Translation: AAH52301.2 Different initiation.
BC063817 mRNA Translation: AAH63817.2 Different initiation.
AY221751 mRNA Translation: AAO67709.1 Different initiation.
AY304473 mRNA Translation: AAQ74770.1 Different initiation.
AF130049 mRNA Translation: AAG35477.1 Different initiation.
EF011612 mRNA Translation: ABK41102.1 Different initiation.
AK024669 mRNA Translation: BAB14955.1 Sequence problems.
CCDSiCCDS31037.2 [Q9H7D7-1]
RefSeqiNP_001108585.2, NM_001115113.2 [Q9H7D7-2]
NP_079436.4, NM_025160.6 [Q9H7D7-1]
UniGeneiHs.497873
Hs.729289

Genome annotation databases

EnsembliENST00000414423; ENSP00000408108; ENSG00000162923 [Q9H7D7-1]
GeneIDi80232
KEGGihsa:80232
UCSCiuc001hop.4 human [Q9H7D7-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB209887 mRNA Translation: BAD93124.1 Different initiation.
AC099790 Genomic DNA No translation available.
BC031471 mRNA Translation: AAH31471.2
BC034498 mRNA Translation: AAH34498.1
BC052301 mRNA Translation: AAH52301.2 Different initiation.
BC063817 mRNA Translation: AAH63817.2 Different initiation.
AY221751 mRNA Translation: AAO67709.1 Different initiation.
AY304473 mRNA Translation: AAQ74770.1 Different initiation.
AF130049 mRNA Translation: AAG35477.1 Different initiation.
EF011612 mRNA Translation: ABK41102.1 Different initiation.
AK024669 mRNA Translation: BAB14955.1 Sequence problems.
CCDSiCCDS31037.2 [Q9H7D7-1]
RefSeqiNP_001108585.2, NM_001115113.2 [Q9H7D7-2]
NP_079436.4, NM_025160.6 [Q9H7D7-1]
UniGeneiHs.497873
Hs.729289

3D structure databases

ProteinModelPortaliQ9H7D7
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123195, 94 interactors
CORUMiQ9H7D7
IntActiQ9H7D7, 38 interactors
MINTiQ9H7D7
STRINGi9606.ENSP00000408108

PTM databases

iPTMnetiQ9H7D7
PhosphoSitePlusiQ9H7D7

Polymorphism and mutation databases

BioMutaiWDR26
DMDMi134047967

Proteomic databases

EPDiQ9H7D7
MaxQBiQ9H7D7
PaxDbiQ9H7D7
PeptideAtlasiQ9H7D7
PRIDEiQ9H7D7
ProteomicsDBi81109
81110 [Q9H7D7-2]
81111 [Q9H7D7-3]
81112 [Q9H7D7-4]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000414423; ENSP00000408108; ENSG00000162923 [Q9H7D7-1]
GeneIDi80232
KEGGihsa:80232
UCSCiuc001hop.4 human [Q9H7D7-1]

Organism-specific databases

CTDi80232
DisGeNETi80232
EuPathDBiHostDB:ENSG00000162923.14
GeneCardsiWDR26
HGNCiHGNC:21208 WDR26
HPAiHPA028539
HPA061094
MalaCardsiWDR26
MIMi617424 gene
617616 phenotype
neXtProtiNX_Q9H7D7
OpenTargetsiENSG00000162923
Orphaneti513456 Intellectual disability-seizures-abnormal gait-facial dysmorphism syndrome
PharmGKBiPA134907873
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0293 Eukaryota
ENOG410XQ2U LUCA
GeneTreeiENSGT00730000111021
HOVERGENiHBG083958
InParanoidiQ9H7D7
KOiK22382
OMAiQCLYHNT
OrthoDBiEOG091G05NI
PhylomeDBiQ9H7D7
TreeFamiTF314869

Enzyme and pathway databases

SignaLinkiQ9H7D7

Miscellaneous databases

ChiTaRSiWDR26 human
GeneWikiiWDR26
GenomeRNAii80232
PROiPR:Q9H7D7
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000162923 Expressed in 225 organ(s), highest expression level in sperm
CleanExiHS_WDR26
ExpressionAtlasiQ9H7D7 baseline and differential
GenevisibleiQ9H7D7 HS

Family and domain databases

Gene3Di2.130.10.10, 1 hit
InterProiView protein in InterPro
IPR006595 CTLH_C
IPR006594 LisH
IPR015943 WD40/YVTN_repeat-like_dom_sf
IPR001680 WD40_repeat
IPR017986 WD40_repeat_dom
IPR036322 WD40_repeat_dom_sf
PfamiView protein in Pfam
PF00400 WD40, 4 hits
SMARTiView protein in SMART
SM00668 CTLH, 1 hit
SM00320 WD40, 5 hits
SUPFAMiSSF50978 SSF50978, 1 hit
PROSITEiView protein in PROSITE
PS50897 CTLH, 1 hit
PS50896 LISH, 1 hit
PS50082 WD_REPEATS_2, 3 hits
PS50294 WD_REPEATS_REGION, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiWDR26_HUMAN
AccessioniPrimary (citable) accession number: Q9H7D7
Secondary accession number(s): A0MNN3
, Q4G100, Q59EC4, Q5GLZ9, Q86UY4, Q9H3C2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 24, 2004
Last sequence update: March 20, 2007
Last modified: November 7, 2018
This is version 160 of the entry and version 3 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

Documents

  1. Human chromosome 1
    Human chromosome 1: 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
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