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FLASH GENE
Symbol MTERF3 contributors: mct - updated : 03-10-2014
HGNC name mitochondrial transcription termination factor 3
HGNC id 24258
Location 8q22.1      Physical location : -
Genatlas name mitochondrial termination factor 1
Synonym name
  • mTERF domain-containing protein 1, mitochondrial
  • MTERF domain containing 1
  • Synonym symbol(s) CGI-12, FLJ10939, MTERFD1
    DNA
    TYPE functioning gene
    STRUCTURE 22.22 kb     8 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 - 1424 48 417 - 2010 20550934
    9 - 1532 - 361 - 2010 20550934
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularvessel   highly
    Endocrineadrenal gland   highly
    Reproductivefemale systemuteruscervix moderately
    Respiratoryrespiratory tractlarynx  predominantly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a 270 AAs long MTERF-domain required for DNA binding and transcription regulation
  • repetitions of a 30 AA module, the mTERF motif, containing leucine zipper-like heptads b
  • 8 MTERF motifs and an additional distorted motif in the C terminus
  • secondary structure MTERF-motif containing three alpha-helices separated by loops
    HOMOLOGY
    interspecies homolog to rattus Mterfd1 (78.2 pc)
    homolog to murine Mterfd1 (76.9 pc)
    Homologene
    FAMILY
  • mTERF family
  • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    basic FUNCTION
  • acting as a negative regulator of mtDNA transcription initiation
  • can mediate the formation of a loop in mitochondrial DNA to promote efficient rRNA synthesis
  • with MTERFD2, and MTERFD3 regulate transcription by acting in the same site in the mtDNA promoter region and thereby mediate fine-tuning of mitochondrial transcription and hence OXPHOS function
  • with MTERFD3 are known to participate in the control of mitochondrial DNA transcription
  • involved in restraining replication fork progression, contributing to genome stability
  • implicated in modulating transcription, replication and protein synthesis
  • novel and unexpected role for MTERF3 in coordinating the crosstalk between transcription and translation for the regulation of mammalian mtDNA gene expression
  • negative regulator of mammalian mitochondrial DNA transcription
  • CELLULAR PROCESS nucleotide, transcription, initiation
    nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA interact with DNA in a sequence-independent manner
    RNA
    small molecule
    protein
  • preferentially interacts with one of the two strands in the double-stranded DNA helix
  • binds to the double-stranded substrate containing the termination sequence
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --over  
    of MTERF3 or MTERFD3 impairs the completion of mitochondrial DNA replication
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS