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Protein

Rho-related GTP-binding protein RhoH

Gene

RHOH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi11 – 188GTPBy similarity
Nucleotide bindingi58 – 625GTPBy similarity
Nucleotide bindingi116 – 1194GTPBy similarity

GO - Molecular functioni

  • 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 GTPase activity Source: GOC
  • negative regulation of I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
  • negative regulation of phosphorylation Source: GOC
  • regulation of small GTPase mediated signal transduction Source: Reactome
  • regulation of transcription, DNA-templated Source: UniProtKB
  • small GTPase mediated signal transduction Source: Reactome
  • T cell differentiation Source: UniProtKB
Complete GO annotation...

Keywords - Ligandi

GTP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi3.6.5.2. 2681.
ReactomeiREACT_11051. 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
ProteomesiUP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:686. RHOH.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: UniProtKB
  • cytosol Source: Reactome
  • immunological synapse Source: Ensembl
  • plasma membrane Source: Reactome
Complete GO annotation...

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

Orphaneti324294. T-cell immunodeficiency with epidermodysplasia verruciformis.
PharmGKBiPA24979.

Polymorphism and mutation databases

DMDMi2500200.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 188188Rho-related GTP-binding protein RhoHPRO_0000198867Add
BLAST
Propeptidei189 – 1913Removed in mature formBy similarityPRO_0000281218

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei188 – 1881Cysteine methyl esterBy similarity
Lipidationi188 – 1881S-geranylgeranyl cysteineBy similarity

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.
PRIDEiQ15669.

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

BgeeiQ15669.
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 PAK7/PAK5 (By similarity).By similarity

Protein-protein interaction databases

BioGridi106892. 6 interactions.
MINTiMINT-1216771.
STRINGi9606.ENSP00000371219.

Structurei

3D structure databases

ProteinModelPortaliQ15669.
SMRiQ15669. Positions 4-171.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni73 – 8614Interaction with ZAP70By similarityAdd
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi33 – 419Effector regionBy similarity

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

eggNOGiCOG1100.
HOGENOMiHOG000233974.
HOVERGENiHBG009351.
InParanoidiQ15669.
KOiK07873.
OMAiQQVFEYA.
OrthoDBiEOG7TJ3K0.
PhylomeDBiQ15669.
TreeFamiTF331219.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
InterProiIPR027417. P-loop_NTPase.
IPR005225. Small_GTP-bd_dom.
IPR001806. Small_GTPase.
IPR003578. Small_GTPase_Rho.
[Graphical view]
PfamiPF00071. Ras. 1 hit.
[Graphical view]
PRINTSiPR00449. RASTRNSFRMNG.
SMARTiSM00174. RHO. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
TIGRFAMsiTIGR00231. small_GTP. 1 hit.
PROSITEiPS51420. RHO. 1 hit.
[Graphical view]

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.
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.
UCSCiuc003guz.2. human.

Keywords - Coding sequence diversityi

Chromosomal rearrangement

Cross-referencesi

Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

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.
UniGeneiHs.654594.
Hs.738915.

3D structure databases

ProteinModelPortaliQ15669.
SMRiQ15669. Positions 4-171.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106892. 6 interactions.
MINTiMINT-1216771.
STRINGi9606.ENSP00000371219.

Polymorphism and mutation databases

DMDMi2500200.

Proteomic databases

PaxDbiQ15669.
PRIDEiQ15669.

Protocols and materials databases

DNASUi399.
Structural Biology KnowledgebaseSearch...

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.
UCSCiuc003guz.2. human.

Organism-specific databases

CTDi399.
GeneCardsiGC04P040192.
HGNCiHGNC:686. RHOH.
HPAiHPA030345.
MIMi602037. gene.
neXtProtiNX_Q15669.
Orphaneti324294. T-cell immunodeficiency with epidermodysplasia verruciformis.
PharmGKBiPA24979.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG1100.
HOGENOMiHOG000233974.
HOVERGENiHBG009351.
InParanoidiQ15669.
KOiK07873.
OMAiQQVFEYA.
OrthoDBiEOG7TJ3K0.
PhylomeDBiQ15669.
TreeFamiTF331219.

Enzyme and pathway databases

BRENDAi3.6.5.2. 2681.
ReactomeiREACT_11051. Rho GTPase cycle.

Miscellaneous databases

ChiTaRSiRHOH. human.
GeneWikiiRhoH.
GenomeRNAii399.
NextBioi1673.
PROiQ15669.
SOURCEiSearch...

Gene expression databases

BgeeiQ15669.
CleanExiHS_RHOH.
ExpressionAtlasiQ15669. baseline and differential.
GenevisibleiQ15669. HS.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
InterProiIPR027417. P-loop_NTPase.
IPR005225. Small_GTP-bd_dom.
IPR001806. Small_GTPase.
IPR003578. Small_GTPase_Rho.
[Graphical view]
PfamiPF00071. Ras. 1 hit.
[Graphical view]
PRINTSiPR00449. RASTRNSFRMNG.
SMARTiSM00174. RHO. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
TIGRFAMsiTIGR00231. small_GTP. 1 hit.
PROSITEiPS51420. RHO. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "TTF, a gene encoding a novel small G protein, fuses to the lymphoma-associated LAZ3 gene by t(3;4) chromosomal translocation."
    Dallery E., Galiegue-Zouitina S., Collyn-D'Hooghe M., Quief S., Denis C., Hildebrand M.-P., Lantoine D., Deweindt C., Tilly H., Bastard C., Kerckaert J.-P.
    Oncogene 10:2171-2178(1995) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  2. "cDNA clones of human proteins involved in signal transduction sequenced by the Guthrie cDNA resource center (www.cdna.org)."
    Puhl H.L. III, Ikeda S.R., Aronstam R.S.
    Submitted (APR-2002) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: B-cell.
  4. "The hematopoiesis-specific GTP-binding protein RhoH is GTPase deficient and modulates activities of other Rho GTPases by an inhibitory function."
    Li X., Bu X., Lu B., Avraham H., Flavell R.A., Lim B.
    Mol. Cell. Biol. 22:1158-1171(2002) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, INTERACTION WITH GDI1 AND GDI2, SUBCELLULAR LOCATION, INDUCTION, TISSUE SPECIFICITY.
  5. "Multiple Rho proteins regulate the subcellular targeting of PAK5."
    Wu X., Frost J.A.
    Biochem. Biophys. Res. Commun. 351:328-335(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH PAK7.
  6. Cited for: FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, INDUCTION.

Entry informationi

Entry nameiRHOH_HUMAN
AccessioniPrimary (citable) accession number: Q15669
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1997
Last modified: June 24, 2015
This is version 150 of the entry and version 1 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

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

External Data

Dasty 3

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 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.