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Protein

Rho-related GTP-binding protein RhoH

Gene

RHOH

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

Functioni

Negative regulator of hematopoietic progenitor cell proliferation, survival and migration. Critical regulator of thymocyte development and T-cell antigen receptor (TCR) signaling by mediating recruitment and activation of ZAP70. Required for phosphorylation of CD3Z, membrane translocation of ZAP70 and subsequent activation of the ZAP70-mediated pathways. Essential for efficient beta-selection and positive selection by promoting the ZAP70-dependent phosphorylation of the LAT signalosome during pre-TCR and TCR signaling. Crucial for thymocyte maturation during DN3 to DN4 transition and during positive selection. Plays critical roles in mast cell function by facilitating phosphorylation of SYK in Fc epsilon RI-mediated signal transduction. Essential for the phosphorylation of LAT, LCP2, PLCG1 and PLCG2 and for Ca2+ mobilization in mast cells (By similarity). Binds GTP but lacks intrinsic GTPase activity and is resistant to Rho-specific GTPase-activating proteins. Inhibits the activation of NF-kappa-B by TNF and IKKB and the activation of CRK/p38 by TNF. Inhibits activities of RAC1, RHOA and CDC42. Negatively regulates leukotriene production in neutrophils.By similarity2 Publications

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi11 – 18GTPBy similarity8
Nucleotide bindingi58 – 62GTPBy similarity5
Nucleotide bindingi116 – 119GTPBy similarity4

GO - Molecular functioni

  • GTPase activity Source: InterPro
  • GTPase inhibitor activity Source: UniProtKB
  • GTP binding Source: UniProtKB
  • kinase inhibitor activity Source: UniProtKB
  • Rho GTPase binding Source: UniProtKB

GO - Biological processi

  • mast cell activation Source: Ensembl
  • negative regulation of I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
  • regulation of small GTPase mediated signal transduction Source: Reactome
  • regulation of transcription, DNA-templated Source: UniProtKB
  • small GTPase mediated signal transduction Source: InterPro
  • T cell differentiation Source: UniProtKB

Keywordsi

LigandGTP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi3.6.5.2 2681
ReactomeiR-HSA-194840 Rho GTPase cycle

Names & Taxonomyi

Protein namesi
Recommended name:
Rho-related GTP-binding protein RhoH
Alternative name(s):
GTP-binding protein TTF
Translocation three four protein
Gene namesi
Name:RHOH
Synonyms:ARHH, TTF
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

EuPathDBiHostDB:ENSG00000168421.12
HGNCiHGNC:686 RHOH
MIMi602037 gene
neXtProtiNX_Q15669

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

Cell membrane, Cytoplasm, Membrane

Pathology & Biotechi

Involvement in diseasei

A chromosomal aberration involving RHOH is found in a non-Hodgkin lymphoma cell line. Translocation t(3;4)(q27;p11) with BCL6.

Organism-specific databases

DisGeNETi399
MalaCardsiRHOH
OpenTargetsiENSG00000168421
Orphaneti324294 T-cell immunodeficiency with epidermodysplasia verruciformis
PharmGKBiPA24979

Polymorphism and mutation databases

DMDMi2500200

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001988671 – 188Rho-related GTP-binding protein RhoHAdd BLAST188
PropeptideiPRO_0000281218189 – 191Removed in mature formBy similarity3

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei188Cysteine methyl esterBy similarity1
Lipidationi188S-geranylgeranyl cysteineBy similarity1

Post-translational modificationi

Phosphorylated on tyrosine by LCK. Phosphorylated by FYN. Phosphorylation enhances the interactions with ZAP70 and SYK and is critical for its function in thymocyte development (By similarity).By similarity

Keywords - PTMi

Lipoprotein, Methylation, Phosphoprotein, Prenylation

Proteomic databases

PaxDbiQ15669
PeptideAtlasiQ15669
PRIDEiQ15669

PTM databases

iPTMnetiQ15669
PhosphoSitePlusiQ15669

Expressioni

Tissue specificityi

Expressed only in hematopoietic cells. Present at very high levels in the thymus, less abundant in the spleen, and least abundant in the bone marrow. Expressed at a higher level in the TH1 subtype of T-helper cells than in the TH2 subpopulation. Expressed in neutrophils under inflammatory conditions, such as cystic fibrosis, ulcerative colitis and appendicitis.2 Publications

Inductioni

By CSF2/GM-CSF. Down-regulated by phorbol myristate acetate (PMA).2 Publications

Gene expression databases

BgeeiENSG00000168421
CleanExiHS_RHOH
ExpressionAtlasiQ15669 baseline and differential
GenevisibleiQ15669 HS

Organism-specific databases

HPAiHPA030345

Interactioni

Subunit structurei

Interacts with ZAP70 (via SH2 domains) and the interaction is enhanced by its phosphorylation by LCK. Interacts with SYK and the interaction is enhanced by its phosphorylation by FYN (By similarity). Interacts with GDI1 and GDI2. Interacts with PAK5 (By similarity).By similarity

GO - Molecular functioni

  • Rho GTPase binding Source: UniProtKB

Protein-protein interaction databases

BioGridi106892, 5 interactors
MINTiQ15669
STRINGi9606.ENSP00000371219

Structurei

3D structure databases

ProteinModelPortaliQ15669
SMRiQ15669
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni73 – 86Interaction with ZAP70By similarityAdd BLAST14

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi33 – 41Effector regionBy similarity9

Domaini

The region involved in interaction with ZAP70 is a non-canonical immunoreceptor tyrosine-based activation motif (ITAM).By similarity

Sequence similaritiesi

Belongs to the small GTPase superfamily. Rho family.Curated

Phylogenomic databases

eggNOGiKOG0393 Eukaryota
COG1100 LUCA
GeneTreeiENSGT00760000118978
HOGENOMiHOG000233974
HOVERGENiHBG009351
InParanoidiQ15669
KOiK07873
OMAiCINAIEG
OrthoDBiEOG091G0KVT
PhylomeDBiQ15669
TreeFamiTF331219

Family and domain databases

InterProiView protein in InterPro
IPR027417 P-loop_NTPase
IPR005225 Small_GTP-bd_dom
IPR001806 Small_GTPase
IPR003578 Small_GTPase_Rho
PfamiView protein in Pfam
PF00071 Ras, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR00231 small_GTP, 1 hit
PROSITEiView protein in PROSITE
PS51420 RHO, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q15669-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLSSIKCVLV GDSAVGKTSL LVRFTSETFP EAYKPTVYEN TGVDVFMDGI
60 70 80 90 100
QISLGLWDTA GNDAFRSIRP LSYQQADVVL MCYSVANHNS FLNLKNKWIG
110 120 130 140 150
EIRSNLPCTP VLVVATQTDQ REMGPHRASC VNAMEGKKLA QDVRAKGYLE
160 170 180 190
CSALSNRGVQ QVFECAVRTA VNQARRRNRR RLFSINECKI F
Length:191
Mass (Da):21,331
Last modified:November 1, 1997 - v1
Checksum:iC5BF8929AD1E59D0
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z35227 mRNA Translation: CAA84538.1
AF498975 mRNA Translation: AAM21122.1
BC014261 mRNA Translation: AAH14261.1
CCDSiCCDS3458.1
PIRiI38367
RefSeqiNP_001265288.1, NM_001278359.1
NP_001265289.1, NM_001278360.1
NP_001265290.1, NM_001278361.1
NP_001265291.1, NM_001278362.1
NP_001265292.1, NM_001278363.1
NP_001265293.1, NM_001278364.1
NP_001265294.1, NM_001278365.1
NP_001265295.1, NM_001278366.1
NP_001265296.1, NM_001278367.1
NP_001265297.1, NM_001278368.1
NP_001265298.1, NM_001278369.1
NP_004301.1, NM_004310.4
XP_011511994.1, XM_011513692.1
XP_016863677.1, XM_017008188.1
XP_016863678.1, XM_017008189.1
UniGeneiHs.654594
Hs.738915

Genome annotation databases

EnsembliENST00000381799; ENSP00000371219; ENSG00000168421
ENST00000505618; ENSP00000425010; ENSG00000168421
ENST00000610353; ENSP00000480192; ENSG00000168421
ENST00000613272; ENSP00000480772; ENSG00000168421
ENST00000614836; ENSP00000478248; ENSG00000168421
ENST00000615083; ENSP00000480379; ENSG00000168421
ENST00000615577; ENSP00000481566; ENSG00000168421
ENST00000617441; ENSP00000482947; ENSG00000168421
ENST00000619474; ENSP00000481746; ENSG00000168421
ENST00000622175; ENSP00000481836; ENSG00000168421
GeneIDi399
KEGGihsa:399

Keywords - Coding sequence diversityi

Chromosomal rearrangement

Similar proteinsi

Entry informationi

Entry nameiRHOH_HUMAN
AccessioniPrimary (citable) accession number: Q15669
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1997
Last modified: May 23, 2018
This is version 174 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: 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. SIMILARITY comments
    Index of protein domains and families

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