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Possible role of mitochondrial permeability transition pore in the pathogenesis of Huntington disease

dc.contributor.authorQuintanilla R.A.
dc.contributor.authorTapia C.
dc.contributor.authorPérez M.J.
dc.date.accessioned2020-09-02T22:26:28Z
dc.date.available2020-09-02T22:26:28Z
dc.date.issued2017
dc.identifier10.1016/j.bbrc.2016.09.054
dc.identifier.citation483, 4, 1078-1083
dc.identifier.issn0006291X
dc.identifier.urihttps://hdl.handle.net/20.500.12728/5929
dc.descriptionHuntington disease (HD) is a devastating neurological disorder that affects the striatum and cortex of patients. HD patients develop progressive motor dysfunction and psychiatric disturbances with gradual dementia. HD is caused by a pathological expansion of CAG repeats in the huntingtin gene that codifies for a protein called huntingtin (Htt), which principal function is not completely understood. Accumulative evidence shows that this pathological expansion modifies Htt function affecting different neuronal targets, including mitochondrial function which is an important factor that contributes to HD. Interestingly, several groups have shown mitochondrial disturbances including calcium handling defects, depolarization, decrease of mitochondrial transport, ATP reduction, and increase of reactive oxygen species (ROS) in cellular and murine HD models. Systematic analysis of this evidence indicates that a mitochondrial structure, the mitochondrial permeability transition pore (mPTP), could be responsible for these changes that affect mitochondria. The mPTP plays an important role in apoptosis and neurodegeneration. It has also been reported to have some physiological functions in heart development and synaptic communication. In HD, the presence of mutant huntingtin (mHtt) activates this mechanism producing a significant compromise of mitochondrial metabolism and bioenergetics. Considering these findings this review explores the evidence that suggests the important role of mPTP in the mitochondrial impairment induced by mHtt, which leads to calcium derangement and contributes to neuronal dysfunction in HD. © 2016 Elsevier Inc.
dc.language.isoen
dc.publisherElsevier B.V.
dc.subjectCalcium
dc.subjectHuntington disease
dc.subjectMitochondria
dc.subjectMitochondrial permeability pore
dc.subjectOxidative stress
dc.subjectcalcium
dc.subjecthuntingtin
dc.subjectlead
dc.subjectmitochondrial permeability transition pore
dc.subjectcarrier protein
dc.subjectHTT protein, human
dc.subjecthuntingtin
dc.subjectmitochondrial permeability transition pore
dc.subjectbioenergy
dc.subjectbrain dysfunction
dc.subjectbrain function
dc.subjectclinical feature
dc.subjecthuman
dc.subjectHuntington chorea
dc.subjectmitochondrial respiration
dc.subjectneuropathology
dc.subjectneurotoxicity
dc.subjectnonhuman
dc.subjectpriority journal
dc.subjectReview
dc.subjectgenetics
dc.subjectHuntington chorea
dc.subjectpathology
dc.subjectpathophysiology
dc.subjectphysiology
dc.subjectHumans
dc.subjectHuntingtin Protein
dc.subjectHuntington Disease
dc.subjectMitochondrial Membrane Transport Proteins
dc.titlePossible role of mitochondrial permeability transition pore in the pathogenesis of Huntington disease
dc.typeReview


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