FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4385202425> ?p ?o ?g. }

• W4385202425 endingPage "106236" @default.
• W4385202425 startingPage "106236" @default.
• W4385202425 abstract "Huntington's disease (HD) is a fatal neurodegenerative disease with limited treatment options. Human and animal studies have suggested that metabolic and mitochondrial dysfunctions contribute to HD pathogenesis. Here, we use high-resolution respirometry to uncover defective mitochondrial oxidative phosphorylation and electron transfer capacity when a mutant huntingtin fragment is targeted to neurons or muscles in Drosophila and find that enhancing mitochondrial function can ameliorate these defects. In particular, we find that co-expression of parkin, an E3 ubiquitin ligase critical for mitochondrial dynamics and homeostasis, produces significant enhancement of mitochondrial respiration when expressed either in neurons or muscles, resulting in significant rescue of neurodegeneration, viability and longevity in HD model flies. Targeting mutant HTT to muscles results in larger mitochondria and higher mitochondrial mass, while co-expression of parkin increases mitochondrial fission and decreases mass. Furthermore, directly addressing HD-mediated defects in the fly's mitochondrial electron transport system, by rerouting electrons to either bypass mitochondrial complex I or complexes III-IV, significantly increases mitochondrial respiration and results in a striking rescue of all phenotypes arising from neuronal mutant huntingtin expression. These observations suggest that bypassing impaired mitochondrial respiratory complexes in HD may have therapeutic potential for the treatment of this devastating disorder." @default.
• W4385202425 created "2023-07-25" @default.
• W4385202425 creator A5009395814 @default.
• W4385202425 creator A5014620806 @default.
• W4385202425 creator A5026475196 @default.
• W4385202425 creator A5030576241 @default.
• W4385202425 creator A5036156104 @default.
• W4385202425 creator A5044563234 @default.
• W4385202425 creator A5054839917 @default.
• W4385202425 creator A5064541607 @default.
• W4385202425 creator A5080158762 @default.
• W4385202425 creator A5082062153 @default.
• W4385202425 creator A5092531515 @default.
• W4385202425 date "2023-09-01" @default.
• W4385202425 modified "2023-09-26" @default.
• W4385202425 title "Bypassing mitochondrial defects rescues Huntington's phenotypes in Drosophila" @default.
• W4385202425 cites W1508962729 @default.
• W4385202425 cites W1550876205 @default.
• W4385202425 cites W1613809967 @default.
• W4385202425 cites W1643390591 @default.
• W4385202425 cites W1809261872 @default.
• W4385202425 cites W1969205203 @default.
• W4385202425 cites W1971753436 @default.
• W4385202425 cites W1972139565 @default.
• W4385202425 cites W1973926398 @default.
• W4385202425 cites W1974769584 @default.
• W4385202425 cites W1976645698 @default.
• W4385202425 cites W1979645469 @default.
• W4385202425 cites W1987732936 @default.
• W4385202425 cites W1994536298 @default.
• W4385202425 cites W1995618419 @default.
• W4385202425 cites W2001163298 @default.
• W4385202425 cites W2003748109 @default.
• W4385202425 cites W2007997420 @default.
• W4385202425 cites W2008584420 @default.
• W4385202425 cites W2010369463 @default.
• W4385202425 cites W2011124366 @default.
• W4385202425 cites W2016507593 @default.
• W4385202425 cites W2017957245 @default.
• W4385202425 cites W2025969862 @default.
• W4385202425 cites W2037640340 @default.
• W4385202425 cites W2038537185 @default.
• W4385202425 cites W2038976751 @default.
• W4385202425 cites W2041248195 @default.
• W4385202425 cites W2041940338 @default.
• W4385202425 cites W2042879634 @default.
• W4385202425 cites W2043078214 @default.
• W4385202425 cites W2046453744 @default.
• W4385202425 cites W2047106689 @default.
• W4385202425 cites W2050347556 @default.
• W4385202425 cites W2050849483 @default.
• W4385202425 cites W2051385624 @default.
• W4385202425 cites W2054556550 @default.
• W4385202425 cites W2055124287 @default.
• W4385202425 cites W2057791111 @default.
• W4385202425 cites W2066290751 @default.
• W4385202425 cites W2069132567 @default.
• W4385202425 cites W2069448970 @default.
• W4385202425 cites W2069477808 @default.
• W4385202425 cites W2070381032 @default.
• W4385202425 cites W2072809920 @default.
• W4385202425 cites W2074111563 @default.
• W4385202425 cites W2075326685 @default.
• W4385202425 cites W2076073424 @default.
• W4385202425 cites W2077298522 @default.
• W4385202425 cites W2087393295 @default.
• W4385202425 cites W2087957743 @default.
• W4385202425 cites W2088657897 @default.
• W4385202425 cites W2091896743 @default.
• W4385202425 cites W2092568560 @default.
• W4385202425 cites W2092648921 @default.
• W4385202425 cites W2097704104 @default.
• W4385202425 cites W2101699102 @default.
• W4385202425 cites W2102430316 @default.
• W4385202425 cites W2104412320 @default.
• W4385202425 cites W2105186030 @default.
• W4385202425 cites W2106012958 @default.
• W4385202425 cites W2106444069 @default.
• W4385202425 cites W2108326484 @default.
• W4385202425 cites W2112884278 @default.
• W4385202425 cites W2118546680 @default.
• W4385202425 cites W2121132431 @default.
• W4385202425 cites W2121760835 @default.
• W4385202425 cites W2130929178 @default.
• W4385202425 cites W2134401364 @default.
• W4385202425 cites W2135189917 @default.
• W4385202425 cites W2137651420 @default.
• W4385202425 cites W2143161560 @default.
• W4385202425 cites W2143588920 @default.
• W4385202425 cites W2146760369 @default.
• W4385202425 cites W2147158491 @default.
• W4385202425 cites W2150852495 @default.
• W4385202425 cites W2153353641 @default.
• W4385202425 cites W2155015733 @default.
• W4385202425 cites W2155804262 @default.
• W4385202425 cites W2160697936 @default.
• W4385202425 cites W2163710163 @default.
• W4385202425 cites W2163833802 @default.

• next