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Protein

Rho GTPase-activating protein 26

Gene

ARHGAP26

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

Functioni

GTPase-activating protein for RHOA and CDC42.

GO - Molecular functioni

  • GTPase activator activity Source: Reactome
  • phospholipid binding Source: FlyBase

GO - Biological processi

  • actin cytoskeleton organization Source: UniProtKB
  • nervous system development Source: UniProtKB
  • regulation of small GTPase mediated signal transduction Source: Reactome
  • signal transduction Source: InterPro

Keywordsi

Molecular functionGTPase activation

Enzyme and pathway databases

ReactomeiR-HSA-194840. Rho GTPase cycle.

Names & Taxonomyi

Protein namesi
Recommended name:
Rho GTPase-activating protein 26
Alternative name(s):
GTPase regulator associated with focal adhesion kinase
Oligophrenin-1-like protein
Rho-type GTPase-activating protein 26
Gene namesi
Name:ARHGAP26
Synonyms:GRAF, KIAA0621, OPHN1L
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

HGNCiHGNC:17073. ARHGAP26.

Subcellular locationi

GO - Cellular componenti

Keywords - Cellular componenti

Cell junction, Cytoplasm, Cytoskeleton

Pathology & Biotechi

Involvement in diseasei

Leukemia, juvenile myelomonocytic (JMML)
The gene represented in this entry is involved in disease pathogenesis. A chromosomal translocation t(5;11)(q31;q23) with KMT2A/MLL1 has been found in leukemic cells from JMML patients, also carrying inactivating mutations on the second allele (PubMed:10908648).1 Publication
Disease descriptionAn aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages.
See also OMIM:607785
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_013623417N → S in JMML; somatic mutation. 1 PublicationCorresponds to variant dbSNP:rs121918546Ensembl.1

Keywords - Diseasei

Disease mutation, Proto-oncogene

Organism-specific databases

DisGeNETi23092.
MalaCardsiARHGAP26.
MIMi607785. phenotype.
OpenTargetsiENSG00000145819.
PharmGKBiPA134946198.

Polymorphism and mutation databases

BioMutaiARHGAP26.
DMDMi21759332.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000567181 – 814Rho GTPase-activating protein 26Add BLAST814

Proteomic databases

EPDiQ9UNA1.
PaxDbiQ9UNA1.
PeptideAtlasiQ9UNA1.
PRIDEiQ9UNA1.

PTM databases

iPTMnetiQ9UNA1.
PhosphoSitePlusiQ9UNA1.
SwissPalmiQ9UNA1.

Expressioni

Gene expression databases

BgeeiENSG00000145819.
CleanExiHS_ARHGAP26.
ExpressionAtlasiQ9UNA1. baseline and differential.
GenevisibleiQ9UNA1. HS.

Organism-specific databases

HPAiHPA035106.
HPA035107.

Interactioni

Subunit structurei

Interacts with NYAP1, NYAP2 and MYO16 (By similarity). Binds to the C-terminus of PTK2/FAK1.By similarity

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi116720. 15 interactors.
IntActiQ9UNA1. 8 interactors.
MINTiMINT-203139.
STRINGi9606.ENSP00000274498.

Structurei

Secondary structure

1814
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi761 – 765Combined sources5
Beta strandi771 – 774Combined sources4
Beta strandi782 – 785Combined sources4
Beta strandi794 – 801Combined sources8
Beta strandi803 – 807Combined sources5
Helixi808 – 810Combined sources3
Beta strandi811 – 813Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1UGVNMR-A756-814[»]
ProteinModelPortaliQ9UNA1.
SMRiQ9UNA1.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9UNA1.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini265 – 369PHPROSITE-ProRule annotationAdd BLAST105
Domaini383 – 568Rho-GAPPROSITE-ProRule annotationAdd BLAST186
Domaini756 – 814SH3PROSITE-ProRule annotationAdd BLAST59

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi584 – 701Ser-richAdd BLAST118

Keywords - Domaini

SH3 domain

Phylogenomic databases

eggNOGiKOG1451. Eukaryota.
ENOG410YJPS. LUCA.
GeneTreeiENSGT00890000139336.
HOGENOMiHOG000018767.
HOVERGENiHBG067993.
InParanoidiQ9UNA1.
KOiK20071.
OMAiKDFGDFK.
OrthoDBiEOG091G01TT.
PhylomeDBiQ9UNA1.
TreeFamiTF316851.

Family and domain databases

CDDicd07636. BAR_GRAF. 1 hit.
cd12064. SH3_GRAF. 1 hit.
Gene3Di2.30.29.30. 1 hit.
InterProiView protein in InterPro
IPR027267. AH/BAR-dom.
IPR030061. GRAF.
IPR035483. GRAF_BAR.
IPR035481. GRAF_SH3.
IPR011993. PH_dom-like.
IPR001849. PH_domain.
IPR008936. Rho_GTPase_activation_prot.
IPR000198. RhoGAP_dom.
IPR001452. SH3_domain.
PANTHERiPTHR12552:SF7. PTHR12552:SF7. 1 hit.
PfamiView protein in Pfam
PF00169. PH. 1 hit.
PF00620. RhoGAP. 1 hit.
PF14604. SH3_9. 1 hit.
SMARTiView protein in SMART
SM00233. PH. 1 hit.
SM00324. RhoGAP. 1 hit.
SM00326. SH3. 1 hit.
SUPFAMiSSF103657. SSF103657. 1 hit.
SSF48350. SSF48350. 1 hit.
SSF50044. SSF50044. 2 hits.
SSF50729. SSF50729. 1 hit.
PROSITEiView protein in PROSITE
PS50003. PH_DOMAIN. 1 hit.
PS50238. RHOGAP. 1 hit.
PS50002. SH3. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: Q9UNA1-1) [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
MGLPALEFSD CCLDSPHFRE TLKSHEAELD KTNKFIKELI KDGKSLISAL
60 70 80 90 100
KNLSSAKRKF ADSLNEFKFQ CIGDAETDDE MCIARSLQEF ATVLRNLEDE
110 120 130 140 150
RIRMIENASE VLITPLEKFR KEQIGAAKEA KKKYDKETEK YCGILEKHLN
160 170 180 190 200
LSSKKKESQL QEADSQVDLV RQHFYEVSLE YVFKVQEVQE RKMFEFVEPL
210 220 230 240 250
LAFLQGLFTF YHHGYELAKD FGDFKTQLTI SIQNTRNRFE GTRSEVESLM
260 270 280 290 300
KKMKENPLEH KTISPYTMEG YLYVQEKRHF GTSWVKHYCT YQRDSKQITM
310 320 330 340 350
VPFDQKSGGK GGEDESVILK SCTRRKTDSI EKRFCFDVEA VDRPGVITMQ
360 370 380 390 400
ALSEEDRRLW MEAMDGREPV YNSNKDSQSE GTAQLDSIGF SIIRKCIHAV
410 420 430 440 450
ETRGINEQGL YRIVGVNSRV QKLLSVLMDP KTASETETDI CAEWEIKTIT
460 470 480 490 500
SALKTYLRML PGPLMMYQFQ RSFIKAAKLE NQESRVSEIH SLVHRLPEKN
510 520 530 540 550
RQMLQLLMNH LANVANNHKQ NLMTVANLGV VFGPTLLRPQ EETVAAIMDI
560 570 580 590 600
KFQNIVIEIL IENHEKIFNT VPDMPLTNAQ LHLSRKKSSD SKPPSCSERP
610 620 630 640 650
LTLFHTVQST EKQEQRNSII NSSLESVSSN PNSILNSSSS LQPNMNSSDP
660 670 680 690 700
DLAVVKPTRP NSLPPNPSPT SPLSPSWPMF SAPSSPMPTS STSSDSSPVR
710 720 730 740 750
SVAGFVWFSV AAVVLSLARS SLHAVFSLLV NFVPCHPNLH LLFDRPEEAV
760 770 780 790 800
HEDSSTPFRK AKALYACKAE HDSELSFTAG TVFDNVHPSQ EPGWLEGTLN
810
GKTGLIPENY VEFL
Length:814
Mass (Da):92,235
Last modified:May 1, 2000 - v1
Checksum:i5C81DBDECB32B18A
GO
Isoform 2 (identifier: Q9UNA1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     700-754: Missing.

Show »
Length:759
Mass (Da):86,165
Checksum:i8FA48FDF1DCAFB7F
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti355E → G in CAA71414 (PubMed:10908648).Curated1
Sequence conflicti355E → G in CAC29145 (Ref. 3) Curated1
Sequence conflicti355E → G in CAC29146 (Ref. 3) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_013623417N → S in JMML; somatic mutation. 1 PublicationCorresponds to variant dbSNP:rs121918546Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_001659700 – 754Missing in isoform 2. 1 PublicationAdd BLAST55

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF141884 mRNA. Translation: AAD39482.1.
Y10388 Genomic DNA. Translation: CAA71414.2.
CH471062 Genomic DNA. Translation: EAW61876.1.
BC068555 mRNA. Translation: AAH68555.1.
AJ309466
, AJ309467, AJ309468, AJ309469, AJ309470, AJ309471, AJ309472, AJ309473, AJ309474, AJ309475, AJ309476, AJ309477, AJ309478, AJ309479, AJ309480, AJ309481, AJ309482, AJ309483, AJ309484, AJ309485, AJ309486, AJ309487 Genomic DNA. Translation: CAC29145.2.
AJ309466
, AJ309467, AJ309468, AJ309469, AJ309470, AJ309471, AJ309472, AJ309473, AJ309474, AJ309475, AJ309476, AJ309477, AJ309478, AJ309479, AJ309480, AJ309481, AJ309482, AJ309483, AJ309484, AJ309485, AJ309487 Genomic DNA. Translation: CAC29146.2.
AB014521 mRNA. Translation: BAA31596.1.
CCDSiCCDS4277.1. [Q9UNA1-1]
CCDS47297.1. [Q9UNA1-2]
PIRiF59430.
RefSeqiNP_001129080.1. NM_001135608.1. [Q9UNA1-2]
NP_055886.1. NM_015071.4. [Q9UNA1-1]
UniGeneiHs.654668.

Genome annotation databases

EnsembliENST00000274498; ENSP00000274498; ENSG00000145819. [Q9UNA1-1]
ENST00000378004; ENSP00000367243; ENSG00000145819. [Q9UNA1-2]
GeneIDi23092.
KEGGihsa:23092.
UCSCiuc003lmt.4. human. [Q9UNA1-1]

Keywords - Coding sequence diversityi

Alternative splicing, Chromosomal rearrangement

Similar proteinsi

Entry informationi

Entry nameiRHG26_HUMAN
AccessioniPrimary (citable) accession number: Q9UNA1
Secondary accession number(s): O75117
, Q5D035, Q9BYS6, Q9BYS7, Q9UJ00
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 11, 2002
Last sequence update: May 1, 2000
Last modified: August 30, 2017
This is version 170 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 5
    Human chromosome 5: 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