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FLASH GENE
Symbol TRIM54 contributors: mct/ - updated : 31-03-2007
HGNC name tripartite motif-containing 54
HGNC id 16008
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a conserved N-terminal Ring finger motif
  • a zinc-binding type-2 B-box motif
  • a coiled coil dimerization motif box in the central region (RBCC)
  • a COS (C-terminal subgroup one signature) domain
  • a tripartite motif (TRIM)
    • conjugated MetalloP
    HOMOLOGY
    interspecies homolog to murine Rnf30
    Homologene
    FAMILY
  • C-II TRIM family
  • MURF subfamily
  • RBCC family
    • CATEGORY regulatory , DNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule
    intracellular,nucleus,chromatin/chromosome
    text colocalized with microtubules and Z-lines in skeletal muscles
    basic FUNCTION
  • playing a potential role in skeletal myoblast differentiation and myotube fusion
  • affecting microtubules stabiliting
  • striated muscle-restricted components of the sarcomere that are thought to possess ubiquitin ligase activity
  • playing an important role in maintaining cardiac integrity and function after acute myocardial infarction
    • CELLULAR PROCESS cell cycle, division
    cell life, differentiation
    PHYSIOLOGICAL PROCESS development
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA binding
    RNA
    small molecule metal binding,
  • Zn2+
    • protein
  • interacting with FHL2 and gamma-filamin (controlling the degradation of FHL2 and FLNC)
    • cell & other
  • microtubules
    • REGULATION
    Other upregulated during striated muscle development
    regulated by IGF2BP2 (regulates the expression of LIMS2 and TRIM54 proteins via direct binding to their mRNAs)
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional        
    resulting in accumulation of FHL2 and FLNC, widening of the sarcomere, diminished contractility and susceptibility to ventricular rupture
    Susceptibility to cardiac rupture after myocardial infarction
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS