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FLASH GENE
Symbol TOP1MT contributors: mct - updated : 30-11-2015
HGNC name topoisomerase (DNA) I, mitochondrial
HGNC id 29787
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
HOMOLOGY
interspecies homolog to Drosophila top1
ortholog to murine Top1mt
ortholog to rattus top1mt
Homologene
FAMILY eucaryotic type I topoisomerase family
CATEGORY enzyme , DNA associated
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,matrix
intracellular,nucleus
text
  • three different topoisomerases have been identified in mitochondria: TOP1MT, TOP3A and a truncated form of TOP2B
    • basic FUNCTION
  • DNA topoisomerase, type IB, DNA single strand break, binding covalently to the 3' end of the break
  • controling and altering the topologic states of DNA during transcription
  • implicated for mitochondrial integrity and energy metabolism
  • relaxes mitochondrial DNA (mtDNA) supercoiling by introducing transient cleavage complexes wherein the broken DNA strand swivels around the intact strand
  • is required for normal mtDNA homeostasis and for linking mtDNA expansion with hepatocyte proliferation
    • CELLULAR PROCESS nucleotide, chromatin organization, remodeling
    nucleotide, replication
    nucleotide, recombination
    nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA binding to single strand
    RNA
    small molecule metal binding,
  • calcium Ca2+
  • magnesium Mg2+
    • protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Top1mt -/- murine embryonic fibroblasts (MEF) exhibit dysfunctional mitochondrial respiration, which leads decreased ATP production and compensation by increased glycolysis and fatty acid oxidation