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FLASH GENE
Symbol RHOH contributors: mct - updated : 22-10-2011
HGNC name ras homolog gene family, member H
HGNC id 686
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
3 - 1999 - 191 - 1997 9226377
EXPRESSION
Type restricted
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Lymphoid/Immunelymph node   highly
 spleen   highly
 thymus   predominantly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow    Homo sapiens
cells
SystemCellPubmedSpeciesStageRna symbol
Lymphoid/ImmuneB cell Homo sapiens
Lymphoid/ImmuneT cell Homo sapiens
cell lineage hemopoietic cells
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
small RHO-like G protein with GTPase structural motifs
conjugated LipoP
HOMOLOGY
interspecies ortholog to murine Rhoh
ortholog to rattus Rhoh_predicted
Homologene
FAMILY
  • small GTPase superfamily
  • Rho family
  • CATEGORY regulatory , signaling
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,cytosolic
    basic FUNCTION
  • required for maintenance of lymphocyte LFA1 in a nonadhesive state
  • potentially important for pre-TCR and TCR signaling because it allows the efficient interaction of ZAP70 with the LAT signalosome, thus regulating thymocyte development
  • regulates FCER1A signaling in mast cells by facilitating SYK activation, possibly as an adaptor molecule for SYK
  • modulates IL3 signalling through regulation of STAT activity and IL3 receptor expression
  • key adapter protein that maintains LCK in the inactive state, contributing to the regulation of both pre-TCR (T cell receptor) and TCR signalling during T-cell development (
  • plays a role in T development
  • regulates TCR signaling via recruitment of ZAP70 and LCK to CD247 in the immunological synapse
  • functioning as an adaptor molecule in TCR signaling pathway
  • lacking GTPase activity
  • CELLULAR PROCESS cell life, differentiation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling signal transduction
    a component
    INTERACTION
    DNA
    RNA
    small molecule nucleotide,
    GTP
    protein
  • binds and modulates LCK, the non-receptor tyrosine kinase crucial in initiating pre-TCR and TCR signallings
  • interacting with JUND that is an inhibitor of RHOH gene expression
  • cell & other
    REGULATION
    Other its function might be regulated by lysosomal degradation of RHOH protein following TCR complex but not BCR activation
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   translocation    
    translocation t(3;4) with BCL6 in non Hodgkin lymphoma (see BCL6) and t(4;14) (p13;q32) in multiple myeloma
    tumoral     --over  
    in primary chronic lymphocytic leukemia and is positively correlated with the protein levels of ZAP70
    tumoral     --low  
    in hairy cell leukemia
    constitutional       loss of function
    leads to T cell defects and persistent Epidermodysplasia verruciformis -HPV infections, suggesting that T cells play a role in the pathogenesis of chronic Epidermodysplasia verruciformis -HPV infections
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    cancerhemopathy 
    RHOH reconstitution represents a potential new approach to the treatment of hairy cell leukemia
    ANIMAL & CELL MODELS
  • Rhoh-null mice exhibited a severe overall T cell defect and abnormally small numbers of circulating beta7-positive cells