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FLASH GENE
Symbol MIEF1 contributors: mct - updated : 30-06-2017
HGNC name mitochondrial elongation factor 1
HGNC id 25979
Location 22q13.1      Physical location : -
Synonym name
  • mitochondrial elongation factor 1
  • Smith-Magenis syndrome chromosome region, candidate 7-like
  • mitochondrial dynamic protein MID51
  • mitochondrial dynamic protein of 51 kDa
  • Synonym symbol(s) RP5-1104E15.5, FLJ20232, HSU79252, MID51, SMCR7L, AltMIEF1, MID51-MP, dJ1104E15.3,
    DNA
    TYPE functioning gene
    STRUCTURE 18.03 kb     6 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    6 - 5730 - 463 - 2016 27660309
    7 - 4712 - 476 - 2016 27660309
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY DNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,outer
    intracellular,cytoplasm,organelle,membrane
    text
  • anchored to the outer mitochondrial membrane
  • basic FUNCTION
  • SMCR7/SMCR7L form foci and rings around mitochondria similar to the fission mediator dynamin-related protein 1 (DNM1L)
  • SMCR7/SMCR7L are new mediators of mitochondrial division affecting DNM1L action at mitochondria
  • actively promotes mitochondrial fusion, but in a manner distinct from mitofusins
  • mitochondrial regulators MIEF1 and MIEF2 exert partially distinct functions in mitochondrial dynamics
  • MIEF1, MIEF2 can act independently of MFF and FIS1 in DNM1L recruitment, suggesting that they provide specificity to the division of mitochondria (PMUID: 23921378)
  • role of DNM1L adaptor proteins MIEF2 and MIEF1 in mitochondrial fission
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacts with and recruits DNM1L to mitochondria in a manner independent of FIS1, MFF and MfN2 (mitofusin 2), but inhibits DNM1L activity, thus executing a negative effect on mitochondrial fission
  • also interacts with FIS1 and elevated FIS1 levels partially reverse the SMCR7L-induced fusion phenotype
  • DNM1L-dependent mitochondrial fission through MIEF1 MIEF2 regulates cristae remodeling during intrinsic apoptosis
  • DNM1L receptors include the paralogues mitochondrial dynamics proteins of 49 and 51 kDa (MIEF1 and MIEF2) and mitochondrial fission factor (MFF)
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS