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FLASH GENE
Symbol LZTR1 contributors: mlc - updated : 25-06-2020
HGNC name leucine-zipper-like transcriptional regulator, 1
HGNC id 6742
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • six N-terminal kelch motifs
  • basic leucine zipper (bZIP) protein with PEST sequences
  • two BTB/POZ domains at the C terminus (the second mediating the binding to the Golgi complex)
    • HOMOLOGY
    Homologene
    FAMILY BTB-kelch superfamily
    CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,endosome
    basic FUNCTION
  • may help stabilize the Gogli complex
  • play important roles during fundamental cellular processes, such as the regulation of cell morphology, migration, and gene expression
  • probable transcriptional regulator that may play a crucial role in embryogenesis
    • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • LZTR1 acts as a conserved regulator of RAS ubiquitination and MAPK pathway activation
  • LZTR1 is a RIT1 interactor, that acts as an adaptor for protein degradation
  • LZTR1 facilitates polyubiquitination and degradation of RAS-GTPases MRAS, HRAS, NRAS, and KRAS
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) NS10
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional   deletion    
    in DiGeorge syndrome
    Susceptibility to an autosomal dominant inherited disorder of multiple schwannomas
    Variant & Polymorphism other
  • germline loss-of-function mutations in LZTR1 predispose to an inherited disorder of multiple schwannomas
    • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Lztr1 deletion in blood vessels of adult mice leads to abnormal vascular leakage