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MGP000208

UniProt Annotations

Entry Information

Gene Name	ras homolog family member H
Protein Entry	RHOH_HUMAN
UniProt ID	Q15669
Species	Human

Comments

Comment type	Description
Disease	Note=A chromosomal aberration involving RHOH is found in a non-Hodgkin lymphoma cell line. Translocation t(3;4)(q27;p11) with BCL6.
Domain	The region involved in interaction with ZAP70 is a non- canonical immunoreceptor tyrosine-based activation motif (ITAM). {ECO:0000250}.
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:0000269\|PubMed:11809807, ECO:0000269\|PubMed:19414807}.
Induction	By CSF2/GM-CSF. Down-regulated by phorbol myristate acetate (PMA). {ECO:0000269\|PubMed:11809807, ECO:0000269\|PubMed:19414807}.
Ptm	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). {ECO:0000250}.
Similarity	Belongs to the small GTPase superfamily. Rho family. {ECO:0000305}.
Subcellular Location	Cytoplasm {ECO:0000269\|PubMed:11809807, ECO:0000269\|PubMed:19414807}. Cell membrane {ECO:0000250}; Lipid- anchor {ECO:0000250}; Cytoplasmic side {ECO:0000250}. Note=Colocalizes together with ZAP70 in the immunological synapse. {ECO:0000250}.
Subunit	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). {ECO:0000250}.
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:0000269\|PubMed:11809807, ECO:0000269\|PubMed:19414807}.
Web Resource	Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/RHOH93.html";