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FLASH GENE
Symbol PPIF contributors: mct - updated : 09-04-2020
HGNC name peptidylprolyl isomerase F (cyclophilin F)
HGNC id 9259
Location 10q22.3      Physical location : 81.107.219 - 81.115.084
Synonym name
  • intracellular receptor for the immune suppressive agent cycloporin A
  • PPIase F
  • cyclophilin 3
  • cyclophilin D
  • cyclophilin F
  • mitochondrial cyclophilin
  • Synonym symbol(s) CYP3, CYPF, CyP-M, Cyp-D, CypD, FLJ90798, MGC117207
    EC.number 5.2.1.8
    DNA
    TYPE functioning gene
    STRUCTURE 7.87 kb     6 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    6 - 2213 - 207 - 2009 19228691
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   highly Homo sapiens
    Digestiveliver   highly Homo sapiens
    Nervousbrainmidbrainsubstantia nigra moderately Homo sapiens
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES Hydrophobic
    STRUCTURE
    motifs/domains
  • a N terminal hydrophobic signal sequence
  • a central PPI and CSA binding domains
  • a C terminal variable region
  • conjugated HemoP
    mono polymer monomer
    HOMOLOGY
    Homologene
    FAMILY immunophilin class M family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,cytosolic
    basic FUNCTION
  • catalyzing the cis-trans isomerization of proline imidic peptide bonds in oligopeptides
  • accelerate the folding of proteins
  • PPIF-dependent mitochondrial permeability transition (mPT), regulates some forms of necrotic death, but not apoptotic death
  • mitochondrial immunophilin and a key positive regulator of the mitochondrial permeability transition (MPT)
  • physiologic function for PPIF and the mitochondrial permeability transition (MPT) in the regulation of starvation-induced autophagy
  • mitochondrial matrix peptidyl-prolyl isomerase known to modulate opening of the mitochondrial permeability transition pore (MPTP)
  • coronavirus-induced neuronal programmed cell death is PPIF dependent and potentially caspase dispensable
  • mitochondrial matrix peptidyl prolyl isomerase and apoptosis regulator
  • directs mitochondria-to-nuclei inflammatory gene expression in normal and tumor cells
  • role for PPIF in modulating protein acetylation
  • activation of PPIF-mediated MPTP opening is associated with the apoptotic pathway and the mitochondrial alteration in retinal ganglion cell death of ischemic retinal injury
  • mitochondrial thioredoxin system controls the redox state of PPIF which, in turn, may act as a regulator of several processes including ROS production and pro-apoptotic factors release
  • regulates mitochondrial metabolism, and likely cell survival, by promoting more efficient electrons flow through the respiratory chain via increased supercomplex formation
  • not only regulates the permeability transition pore (PTP), but also regulates the dynamics of synthasome assembly depending on the bioenergetic state of the mitochondria
  • controls the opening of mitochondrial permeability transition por
  • physiological function of PPIF in dendritic plasticity by acting as a fine-tuner of mitochondrial calcium homeostasis
  • is involved in the regulation of inflammation in aorta
  • mitochondrial PPIF is involved in the inhibitory effect of high-fat diet (HFD) on STAR expression in testes
  • is an important contributor to Pb-induced mitochondrial abnormality
  • CELLULAR PROCESS protein, post translation, folding
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • complexing with PPID SLC25A3, SLC25A5, ANTX to form a mitochondrial permeability transition pore
  • functional component of the mitochondrial permeability transition pore (mPTP) which allows the exchange of ions and solutes between the mitochondrial matrix to induce mitochondrial swelling and activation of cell death pathways
  • INTERACTION
    DNA
    RNA
    small molecule cofactor, nucleotide,
    NADPH
    protein
  • flavoprotein P450 oxidoreductase (POR)
  • cyclosporin A
  • novel function as a BCL2 collaborator and an inhibitor of cytochrome c release from mitochondria independent of the MPT
  • C1QBP appears to act as an endogenous inhibitor of the mitochondrial permeability transition pore, most likely through binding to PPIF, and thus protects cells against oxidative stress
  • C11QBP could directly bind to PPIF
  • HSPD1 is a novel regulator of mitochondrial permeability transition, contributing to a cytoprotective chaperone network that antagonizes PPIF-dependent cell death in tumors
  • role of PPIF-dependent mitochondrial permeability transition pore (mPTP) in Abeta-impaired axonal mitochondrial trafficking
  • interacts with mitochondrial transcription factors B1 and B2 (TFB2M) but not with mitochondrial transcription factor A
  • binds to a number of important proteins in the mitochondrial matrix and inner mitochondrial membrane
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    attenuates mitochondrial perturbation and ameliorates hepatic steatosis
    constitutional       loss of function
    rescues axonal mitochondrial transport in Alzheimer neurons
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    neurologyneurodegenerativealzheimer
    its deficiency rescues axonal mitochondrial transport in Alzheimer's neurons
    metabolism  
    provides a potential strategy to inhibit Pb neurotoxicity
    cardiovascularaquired 
    blocking mitochondrial PPIF enhances vascular resistance to inflammatory injuries
    ANIMAL & CELL MODELS
  • Ppif(-/-) mice exhibit substantially greater cardiac hypertrophy, fibrosis, and reduction in myocardial function in response to pressure overload stimulation than control mice