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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
Location 4p14      Physical location : 40.198.526 - 40.246.281
Synonym name TTF, translocation three four rho-related GTP-binding protein
Synonym symbol(s) ARHH, TTF
DNA
TYPE functioning gene
STRUCTURE 47.75 kb     3 Exon(s)
regulatory sequence Promoter
Binding site   transcription factor
text structure
  • RHOH gene transcription is initiated by three distinct promoter regions designated P1, P2 and P3 that define the 5prime end of exons 1, 2 and 4 respectively (
  • two AP1 (activator protein 1) sites in the minimal promoter region bind JUND (
  • MAPPING cloned Y linked N status confirmed
    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