InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: RHOH

Summary

InnateDB Protein

IDBP-14853.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

RHOH

Protein Name

ras homolog gene family, member H

Synonyms

ARHH; TTF;

Species

Homo sapiens

Ensembl Protein

ENSP00000371219

InnateDB Gene

IDBG-14849 (RHOH)

Protein Structure

UniProt Annotation

Function

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 Ca(2+) 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. {ECO:0000250, ECO:0000269PubMed:11809807, ECO:0000269PubMed:19414807}.

Subcellular Localization

Cytoplasm {ECO:0000269PubMed:11809807, ECO:0000269PubMed:19414807}. Cell membrane {ECO:0000250}; Lipid- anchor {ECO:0000250}; Cytoplasmic side {ECO:0000250}. Note=Colocalizes together with ZAP70 in the immunological synapse. {ECO:0000250}.

Disease Associations

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

Tissue Specificity

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. {ECO:0000269PubMed:11809807, ECO:0000269PubMed:19414807}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 9 experimentally validated interaction(s) in this database.

Experimentally validated
Total	9 [view]
Protein-Protein	9 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0005095	GTPase inhibitor activity
GO:0005515	protein binding
GO:0005525	GTP binding
GO:0017048	Rho GTPase binding
GO:0019210	kinase inhibitor activity

Biological Process

GO:0006184	GTP catabolic process
GO:0006355	regulation of transcription, DNA-templated
GO:0007165	signal transduction
GO:0007264	small GTPase mediated signal transduction
GO:0015031	protein transport
GO:0030217	T cell differentiation
GO:0042326	negative regulation of phosphorylation
GO:0043086	negative regulation of catalytic activity
GO:0043124	negative regulation of I-kappaB kinase/NF-kappaB signaling
GO:0045576	mast cell activation
GO:0051056	regulation of small GTPase mediated signal transduction

Cellular Component

GO:0001772	immunological synapse
GO:0005622	intracellular
GO:0005737	cytoplasm
GO:0005829	cytosol
GO:0005886	plasma membrane
GO:0016020	membrane

Protein Structure and Domains

PDB ID

InterPro

IPR001806 Small GTPase superfamily
IPR003578 Small GTPase superfamily, Rho type
IPR003579 Small GTPase superfamily, Rab type
IPR005225 Small GTP-binding protein domain
IPR006689 Small GTPase superfamily, ARF/SAR type
IPR013684 Mitochondrial Rho-like
IPR020849 Small GTPase superfamily, Ras type
IPR027417 P-loop containing nucleoside triphosphate hydrolase

PFAM

PF00071
PF00025
PF08477

PRINTS

PR00449
PR00328

PIRSF

SMART

SM00174
SM00175
SM00173

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt