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FLASH GENE
Symbol MIPEP contributors: mct - updated : 06-06-2019
HGNC name mitochondrial intermediate peptidase
HGNC id 7104
Corresponding disease
COXPD31 combined oxidative phosphorylation deficiency 31
Location 13q12.12      Physical location : 24.304.328 - 24.463.587
Synonym symbol(s) HMIP, MIP, PMIP, COXPD31
EC.number 3.4.24.59
DNA
TYPE functioning gene
STRUCTURE 159.26 kb     19 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked   status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
19 - 2361 - 713 - 2016 27799064
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart    
Digestivepharynx   highly
Endocrinepancreas    
 parathyroid   highly
Nervousbrainhindbraincerebellum  
 brain    
 spinal cord    
Reproductivefemale systembreastmammary gland highly
Urinarykidney    
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Muscularstriatumskeletal  
Nervouscentral   
Nervousperipherous   
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
conjugated MetalloP
mono polymer monomer
HOMOLOGY
interspecies homolog to C.elegans f32b6,2
homolog to Rattus norvegicus mitochondrial intermediate peptidase
Homologene
FAMILY peptidase family m3
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,organelle,mitochondria,matrix
basic FUNCTION
  • mitochondrial intermediate peptidase
  • involved in the biogenesis of the oxidative phosphorylation system
  • metalloendopeptidase
  • both BCL11A and MIPEP were associated with increased endogenous levels of HbF
  • is a novel regulator of SIRT3 activation by caloric restriction
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • gamma-secretase-regulated proteolysis of the Notch receptor by MIPEP
  • MIPEP may contribute to the maintenance of mitochondrial quality during caloric restriction (CR) via activation of SIRT3
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) COXPD31
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS