Matches in SemOpenAlex for { <https://semopenalex.org/work/W2521004562> ?p ?o ?g. }
- W2521004562 endingPage "1083" @default.
- W2521004562 startingPage "1078" @default.
- W2521004562 abstract "Huntington 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." @default.
- W2521004562 created "2016-09-23" @default.
- W2521004562 creator A5007582714 @default.
- W2521004562 creator A5050126878 @default.
- W2521004562 creator A5078544496 @default.
- W2521004562 date "2017-02-01" @default.
- W2521004562 modified "2023-09-24" @default.
- W2521004562 title "Possible role of mitochondrial permeability transition pore in the pathogenesis of Huntington disease" @default.
- W2521004562 cites W1480051253 @default.
- W2521004562 cites W1489178621 @default.
- W2521004562 cites W1500536335 @default.
- W2521004562 cites W1507443798 @default.
- W2521004562 cites W1546052201 @default.
- W2521004562 cites W1563847798 @default.
- W2521004562 cites W1565788467 @default.
- W2521004562 cites W1650328097 @default.
- W2521004562 cites W1809261872 @default.
- W2521004562 cites W1814641909 @default.
- W2521004562 cites W1905860502 @default.
- W2521004562 cites W1920278102 @default.
- W2521004562 cites W1965703692 @default.
- W2521004562 cites W1966329382 @default.
- W2521004562 cites W1966468660 @default.
- W2521004562 cites W1967561486 @default.
- W2521004562 cites W1981478938 @default.
- W2521004562 cites W1987404072 @default.
- W2521004562 cites W1994473527 @default.
- W2521004562 cites W1994758764 @default.
- W2521004562 cites W2010369463 @default.
- W2521004562 cites W2011035933 @default.
- W2521004562 cites W2014002213 @default.
- W2521004562 cites W2015041659 @default.
- W2521004562 cites W2016888445 @default.
- W2521004562 cites W2020117714 @default.
- W2521004562 cites W2027367467 @default.
- W2521004562 cites W2032105133 @default.
- W2521004562 cites W2033297517 @default.
- W2521004562 cites W2047121554 @default.
- W2521004562 cites W2049113387 @default.
- W2521004562 cites W2052390988 @default.
- W2521004562 cites W2052620246 @default.
- W2521004562 cites W2052737683 @default.
- W2521004562 cites W2053985220 @default.
- W2521004562 cites W2061151435 @default.
- W2521004562 cites W2062682951 @default.
- W2521004562 cites W2067297186 @default.
- W2521004562 cites W2069839583 @default.
- W2521004562 cites W2072396956 @default.
- W2521004562 cites W2074304614 @default.
- W2521004562 cites W2074800090 @default.
- W2521004562 cites W2081949482 @default.
- W2521004562 cites W2083038107 @default.
- W2521004562 cites W2084842110 @default.
- W2521004562 cites W2087157508 @default.
- W2521004562 cites W2096944314 @default.
- W2521004562 cites W2097527232 @default.
- W2521004562 cites W2100103422 @default.
- W2521004562 cites W2113149589 @default.
- W2521004562 cites W2116673443 @default.
- W2521004562 cites W2117600112 @default.
- W2521004562 cites W2121642311 @default.
- W2521004562 cites W2121760835 @default.
- W2521004562 cites W2130508542 @default.
- W2521004562 cites W2131203829 @default.
- W2521004562 cites W2131472300 @default.
- W2521004562 cites W2139826556 @default.
- W2521004562 cites W2142125979 @default.
- W2521004562 cites W2144611722 @default.
- W2521004562 cites W2146267270 @default.
- W2521004562 cites W2157783585 @default.
- W2521004562 cites W2159538843 @default.
- W2521004562 cites W2159546257 @default.
- W2521004562 cites W2162965085 @default.
- W2521004562 cites W2164332878 @default.
- W2521004562 cites W2217527544 @default.
- W2521004562 cites W2283318401 @default.
- W2521004562 cites W2345152850 @default.
- W2521004562 cites W2345712655 @default.
- W2521004562 cites W2403525352 @default.
- W2521004562 doi "https://doi.org/10.1016/j.bbrc.2016.09.054" @default.
- W2521004562 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27638306" @default.
- W2521004562 hasPublicationYear "2017" @default.
- W2521004562 type Work @default.
- W2521004562 sameAs 2521004562 @default.
- W2521004562 citedByCount "29" @default.
- W2521004562 countsByYear W25210045622017 @default.
- W2521004562 countsByYear W25210045622018 @default.
- W2521004562 countsByYear W25210045622019 @default.
- W2521004562 countsByYear W25210045622020 @default.
- W2521004562 countsByYear W25210045622021 @default.
- W2521004562 countsByYear W25210045622022 @default.
- W2521004562 countsByYear W25210045622023 @default.
- W2521004562 crossrefType "journal-article" @default.
- W2521004562 hasAuthorship W2521004562A5007582714 @default.
- W2521004562 hasAuthorship W2521004562A5050126878 @default.
- W2521004562 hasAuthorship W2521004562A5078544496 @default.
- W2521004562 hasConcept C100206155 @default.
- W2521004562 hasConcept C104317684 @default.