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FLASH GENE
Symbol OMA1 contributors: mct - updated : 23-08-2017
HGNC name OMA1 zinc metallopeptidase
HGNC id 2661
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