RHOH

The protein encoded by this gene is a member of the Ras superfamily of small GTPases. Expression of a chimeric transcript of LAZ3 and this gene has been reported as a result of the translocation t(3;4) in non-Hodgkin's lymphomas. This gene encodes a small G-like protein, and unlike most other small G proteins which are expressed ubiquitously, this gene is transcribed only in hemopoietic cells. [provided by RefSeq]

ras homolog gene family, member H

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.

Biological Process actin filament organizationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process cell projection assemblyManual Assertion Based On ExperimentIBA:GO_Central
Biological Process cortical cytoskeleton organizationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process engulfment of apoptotic cellManual Assertion Based On ExperimentIBA:GO_Central
Biological Process establishment or maintenance of cell polarityManual Assertion Based On ExperimentIBA:GO_Central
Biological Process mast cell activationIEA:Ensembl
Biological Process motor neuron axon guidanceManual Assertion Based On ExperimentIBA:GO_Central
Biological Process negative regulation of I-kappaB kinase/NF-kappaB signalingManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of T cell differentiationManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process Rac protein signal transductionManual Assertion Based On ExperimentIBA:GO_Central
Biological Process regulation of actin cytoskeleton organizationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process regulation of cell shapeManual Assertion Based On ExperimentIBA:GO_Central
Biological Process regulation of neutrophil migrationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process regulation of transcription, DNA-templated1 PublicationNAS:UniProtKB
Biological Process T cell differentiation1 PublicationNAS:UniProtKB

Cytoplasm
Cell membrane
Colocalizes together with ZAP70 in the immunological synapse.

Epidermodysplasia verruciformis 4 (EV4):
A form of epidermodysplasia verruciformis, a rare genodermatosis associated with a high risk of skin carcinoma that results from an abnormal susceptibility to infection by specific human papillomaviruses, including the oncogenic HPV5. Infection leads to the early development of disseminated flat wart-like and pityriasis versicolor-like skin lesions. Cutaneous Bowen's carcinomas in situ and invasive squamous cell carcinomas develop in about half of the patients, mainly on sun-exposed skin areas. EV4 patients have decreased number of naive T cells, increased memory and effector T cells, and impaired T-cell receptor signaling. EV4 inheritance is autosomal recessive.

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).