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Impaired mitophagy in Fanconi anemia is dependent on mitochondrial fission
Published in Impact Journals LLC
2016
PMID: 27517150
Volume: 7
   
Issue: 36
Pages: 58065 - 58074
Abstract
Fanconi anemia (FA) is a rare genetic disorder associated with bone-marrow failure, genome instability and cancer predisposition. Recently, we and others have demonstrated dysfunctional mitochondria with morphological alterations in FA cells accompanied by high reactive oxygen species (ROS) levels. Mitochondrial morphology is regulated by continuous fusion and fission events and the misbalance between these two is often accompanied by autophagy. Here, we provide evidence of impaired autophagy in FA. We demonstrate that FA cells have increased number of autophagic (presumably mitophagic) events and accumulate dysfunctional mitochondria due to an impaired ability to degrade them. Moreover, mitochondrial fission accompanied by oxidative stress (OS) is a prerequisite condition for mitophagy in FA and blocking this pathway may release autophagic machinery to clear dysfunctional mitochondria.
About the journal
JournalOncotarget
PublisherImpact Journals LLC
ISSN19492553
Open AccessYes
Concepts (56)
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    DYNAMIN RELATED PROTEIN 1
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    Guanosine triphosphatase
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    HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE KINASE
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    Membrane protein
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    PARKIN
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    PROTEIN PINK1
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    Reactive oxygen metabolite
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    Small interfering rna
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    Unclassified drug
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    Article
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    AUTOLYSOSOME
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    Autophagy
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    Controlled study
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    DISEASE ASSOCIATION
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    DISORDERS OF MITOCHONDRIAL FUNCTIONS
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    Down regulation
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    FANCONI ANEMIA
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    Flow cytometry
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    FLUORESCENCE ACTIVATED CELL SORTING
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    Gene expression regulation
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    Gene silencing
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    Genetic transfection
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    Human
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    Human cell
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    INTRACELLULAR SIGNALING
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    Lysosome
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    MITOCHONDRIAL BIOGENESIS
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    MITOCHONDRIAL DYNAMICS
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    MITOCHONDRIAL FISSION
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    MITOCHONDRIAL VOLUME
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    MITOPHAGY
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    Molecular dynamics
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    Oxidative stress
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    Phenotype
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    Scanning electron microscopy
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    Transmission electron microscopy
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    Upregulation
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    Validation process
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    Cell line
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    FANCONI ANEMIA
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    Fluorescence microscopy
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    Metabolism
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    Mitochondrion
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    Pathology
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    Pathophysiology
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    RARE DISEASE
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    Ultrastructure
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    FANCONI ANEMIA
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    Humans
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    Microscopy, electron, transmission
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    Microscopy, fluorescence
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    Mitochondria
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    MITOCHONDRIAL DEGRADATION
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    MITOCHONDRIAL DYNAMICS
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    RARE DISEASES
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    Reactive oxygen species