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FLASH GENE
Symbol OMA1 contributors: mct - updated : 23-08-2017
HGNC name OMA1 zinc metallopeptidase
HGNC id 2661
Corresponding disease
Location 1p32.2      Physical location : 58.946.391 - 59.012.446
Synonym name
  • metalloprotease-related protein 1
  • overlapping activity with M-AAA protease
  • overlapping with the m-AAA protease 1 homolog
  • zinc metallopeptidase OMA1
    • Synonym symbol(s) MPRP-1, YKR087C, ZMPOMA1
    DNA
    TYPE functioning gene
    STRUCTURE 66.08 kb     9 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 - 1953 - 523 - 2009 20038677
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Reproductivemale systemprostate  highly
     male systemtestis  highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period fetal
    Text
  • predominantly expressed at late developmental stages
    • PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
    conjugated PhosphoP
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY adaptor
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,cytosolic
    text
  • most OMA1 medial septum/diagonal band neurons are probably GABAergic projection neurons
    • basic FUNCTION
  • physiological role for OMA1 protease in fine-tuning of respiratory function
  • is a critical regulator of neuronal survival, demonstrating that stress-induced OPA1 processing by OMA1 promotes neuronal death and neuroinflammatory responses
  • YME1L1 and OMA1 are critical regulators of essential mitochondrial functions, including inner membrane proteostasis maintenance and mitochondrial dynamics
  • ATP-independent metalloprotease
  • mitochondrial protease OMA1 and the poorly characterized protein DELE1, together with EIF2AK1, EIF2AK3, constitute the missing pathway that is triggered by mitochondrial stress
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS development
    PATHWAY
    metabolism
    signaling signal transduction
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease
  • OMA1 peptidase mediates the degradation of long isoforms of the dynamin-like GTPase OPA1 in the inner membrane
  • stress-induced OPA1 processing by OMA1 converts OPA1 completely into short isoforms, inhibits fusion, and triggers mitochondrial fragmentation
  • oligomerized BAX and BAK1 trigger apoptosis by causing both the permeabilization of the mitochondrial outer membrane and activation OMA1
  • differential degradation of YME1L1 and OMA1 alters their proteolytic processing of the dynamin-like GTPase OPA1, a critical regulator of mitochondrial inner membrane morphology, which influences the recovery of tubular mitochondria following membrane-depolarization-induced fragmentation
    • cell & other
    REGULATION
    Other OMA1 is degraded through a YME1L1-dependent mechanism in response to toxic insults that depolarize the mitochondrial membrane
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility to autism
    Variant & Polymorphism SNP
  • variations in OMA1 involved in the Reelin signaling pathway might contribute to genetic susceptibility to autism
    • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mdab1 null mice, scrambler and yotari mice
  • mice deficient in Dab1, Reelin, or the Reelin receptors ApoER2 and VLDLR exhibit severely perturbed brain cytoarchitecture