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FLASH GENE
Symbol RHOT1 contributors: mct - updated : 05-05-2017
HGNC name ras homolog gene family, member T1
HGNC id 21168
Location 17q11.2      Physical location : 30.469.472 - 30.552.745
Synonym name
  • mitochondrial Ras homolog gene family, member T1
  • rac-GTP binding protein-like protein
  • Synonym symbol(s) MIRO-1, FLJ11040, ARHT1, FLJ12633, MIRO1
    EC.number 3.6.5.-
    DNA
    TYPE functioning gene
    STRUCTURE 110.92 kb     21 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    21 - 3309 - 691 - 2016 26658612
    20 - 3213 - 659 - 2016 26658612
    19 - 3089 - 618 - 2016 26658612
    21 - 3360 - 523 - 2016 26658612
    20 - 3264 - 491 - 2016 26658612
    21 - 3236 - 650 - 2016 26658612
    19 - 3136 - 597 - 2016 26658612
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveesophagus   highly
    Lymphoid/Immunethymus   highly
    Reproductivefemale systemuterus   
    Respiratoryrespiratory tractlarynx  highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • tandem GTP-binding domains separated by a linker region with putative calcium-binding motives
  • linker region containing putative calcium-binding EF hand motifs
  • C-terminal transmembrane domain, which confers targeting to the mitochondria
  • HOMOLOGY
    interspecies ortholog to murine Arht1
    Homologene
    FAMILY
  • ras superfamily
  • mitochondrial Rho GTPase family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,mitochondria,outer
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,cytosolic
    basic FUNCTION
  • mitochondrial homeostasis and apoptosis
  • atypical Rho GTPases, having essential roles in mitochondrial trafficking
  • MIRO proteins that serve as a [Ca(2+)](c)-sensitive switch and bifunctional regulator for both the motility and fusion-fission dynamics of the mitochondria
  • RHOT1 and TRAK2, play an important role in regulating mitochondrial transport in neurons
  • RHOT1, RHOT2 are involved in axonal transport of mitochondria in neurons
  • RHOT1, RHOT2 are core components of mitochondrial transport complexes
  • mitochondrial redistribution and efficient lymphocyte adhesion to the endothelium require the function of RHOT1, an adaptor molecule that couples mitochondria to microtubules
  • associates with the dynein complex
  • key role of RHOT1 in the control of lymphocyte adhesion and migration through the regulation of mitochondrial redistribution
  • RhoGTPase which is a key regulator of mitochondrial movement linking mitochondria and motor proteins
  • can regulate cell migration and proliferation by suppressing the expression of SMAD4 in PC (pancreatic cancer), which may provide a novel sight to explore the mechanism and therapeutic strategy for PC
  • RHOT1 and CENPF promote anterograde mitochondrial movement and proper mitochondrial distribution in daughter cells
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule nucleotide,
  • GTP binding
  • calcium Ca2+ binding
  • protein
  • interacts with the Kinesin-binding proteins, TRAK1 and TRAK2, suggesting that the Miro GTPases form a link between the mitochondria and the trafficking apparatus of the microtubules
  • MFN1 and MFN2 interact with mammalian MIRO (RHOT1/RHOT2) and Milton (TRAK1) proteins, members of the molecular complex that links mitochondria to kinesin motors
  • selective binding by TRAK1 and/or TRAK2 to different members of the kinesin transport family or to RHOT1 and RHOT2 may represent crucial regulatory points in controlling the traffic of mitochondrial cargoes in neurons
  • DISC1 associates robustly with TRAK1 which is, in turn, known to interact with the outer mitochondrial membrane proteins RHOT1/2, linking mitochondria to the kinesin motor for microtubule-based subcellular trafficking
  • RHOT1 is a direct PRKN substrate
  • CENPF is recruited to mitochondria by RHOT1 at the time of cytokinesis and associates with microtubule growing tips
  • DISC1 couples to the mitochondrial transport and fusion machinery via interaction with the outer mitochondrial membrane GTPase proteins RHOT1, RHOT2, the TRAK1 and TRAK2 mitochondrial trafficking adaptors, and the mitochondrial fusion proteins (mitofusins)
  • APC interactions with the mitochondrial kinesin-motor complex RHOT1/TRAK2 that were mediated by the APC C-terminus
  • status of RHOT1 phosphorylation influences the decision to undergo Parkin-dependent mitochondrial arrest, which, in the context of PINK1 action on other substrates, can restrict mitochondrial dynamics before mitophagy
  • physical interaction between GBF1 and RHOT1, RHOT2, and also between the active GTP-bound form of ARF1 and RHOT1, RHOT2 (
  • cell & other
    REGULATION
    Phosphorylated by PINK1 (phosphorylation of RHOT2 activates proteasomal degradation of RHOT2 in a Parkin-dependent manner)
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in the spinal cord tissue of ALS patients
    tumoral     --low  
    of RHOT1 and SMAD4 were significantly associated with lymph node metastasis and shorter survival in pancreatic cancer
    Susceptibility
    Variant & Polymorphism SNP
    Candidate gene
    Marker
  • may be considered as a potential novel marker for predicting the outcome in patients with pancreatic cancer
  • Therapy target
    ANIMAL & CELL MODELS