Matches in SemOpenAlex for { <https://semopenalex.org/work/W2045337251> ?p ?o ?g. }
- W2045337251 endingPage "81" @default.
- W2045337251 startingPage "72" @default.
- W2045337251 abstract "Mutations in the Cu/Zn Superoxide Dismutase (SOD1) gene cause an inherited form of ALS with upper and lower motor neuron loss. The mechanism underlying mutant SOD1-mediated motor neuron degeneration remains unclear. While defects in mitochondrial dynamics contribute to neurodegeneration, including ALS, previous reports remain conflicted. Here, we report an improved technique to isolate, transfect, and culture rat spinal cord motor neurons. Using this improved system, we demonstrate that mutant SOD1(G93A) triggers a significant decrease in mitochondrial length and an accumulation of round fragmented mitochondria. The increase of fragmented mitochondria coincides with an arrest in both anterograde and retrograde axonal transport and increased cell death. In addition, mutant SOD1(G93A) induces a reduction in neurite length and branching that is accompanied with an abnormal accumulation of round mitochondria in growth cones. Furthermore, restoration of the mitochondrial fission and fusion balance by dominant-negative dynamin-related protein 1 (DRP1) expression rescues the mutant SOD1(G93A)-induced defects in mitochondrial morphology, dynamics, and cell viability. Interestingly, both SIRT3 and PGC-1α protect against mitochondrial fragmentation and neuronal cell death by mutant SOD1(G93A). This data suggests that impairment in mitochondrial dynamics participates in ALS and restoring this defect might provide protection against mutant SOD1(G93A)-induced neuronal injury." @default.
- W2045337251 created "2016-06-24" @default.
- W2045337251 creator A5009733602 @default.
- W2045337251 creator A5036482012 @default.
- W2045337251 creator A5036690654 @default.
- W2045337251 creator A5048639922 @default.
- W2045337251 creator A5062376265 @default.
- W2045337251 date "2013-03-01" @default.
- W2045337251 modified "2023-09-26" @default.
- W2045337251 title "Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α" @default.
- W2045337251 cites W1762535185 @default.
- W2045337251 cites W1928433275 @default.
- W2045337251 cites W1966132436 @default.
- W2045337251 cites W1970060454 @default.
- W2045337251 cites W1971082641 @default.
- W2045337251 cites W1971397654 @default.
- W2045337251 cites W1972681625 @default.
- W2045337251 cites W1975191791 @default.
- W2045337251 cites W1980114602 @default.
- W2045337251 cites W1982647833 @default.
- W2045337251 cites W1984326148 @default.
- W2045337251 cites W1984979116 @default.
- W2045337251 cites W1985834536 @default.
- W2045337251 cites W1987190216 @default.
- W2045337251 cites W1987738803 @default.
- W2045337251 cites W1995384855 @default.
- W2045337251 cites W1996395636 @default.
- W2045337251 cites W1996934314 @default.
- W2045337251 cites W2004351969 @default.
- W2045337251 cites W2004984537 @default.
- W2045337251 cites W2008584420 @default.
- W2045337251 cites W2018110972 @default.
- W2045337251 cites W2018427433 @default.
- W2045337251 cites W2021419707 @default.
- W2045337251 cites W2022367303 @default.
- W2045337251 cites W2023542615 @default.
- W2045337251 cites W2032142575 @default.
- W2045337251 cites W2037024974 @default.
- W2045337251 cites W2038697027 @default.
- W2045337251 cites W2039374791 @default.
- W2045337251 cites W2040625305 @default.
- W2045337251 cites W2052238208 @default.
- W2045337251 cites W2052751736 @default.
- W2045337251 cites W2068147651 @default.
- W2045337251 cites W2074052524 @default.
- W2045337251 cites W2074740541 @default.
- W2045337251 cites W2079457709 @default.
- W2045337251 cites W2079569030 @default.
- W2045337251 cites W2081818708 @default.
- W2045337251 cites W2084708023 @default.
- W2045337251 cites W2085058400 @default.
- W2045337251 cites W2088381487 @default.
- W2045337251 cites W2088523363 @default.
- W2045337251 cites W2091299458 @default.
- W2045337251 cites W2091489629 @default.
- W2045337251 cites W2092390300 @default.
- W2045337251 cites W2092729353 @default.
- W2045337251 cites W2096880567 @default.
- W2045337251 cites W2097793695 @default.
- W2045337251 cites W2101580126 @default.
- W2045337251 cites W2106819616 @default.
- W2045337251 cites W2107660300 @default.
- W2045337251 cites W2107768418 @default.
- W2045337251 cites W2107846158 @default.
- W2045337251 cites W2109879006 @default.
- W2045337251 cites W2111753882 @default.
- W2045337251 cites W2117427878 @default.
- W2045337251 cites W2118964113 @default.
- W2045337251 cites W2123720975 @default.
- W2045337251 cites W2123779560 @default.
- W2045337251 cites W2125501179 @default.
- W2045337251 cites W2127271204 @default.
- W2045337251 cites W2128207486 @default.
- W2045337251 cites W2129899796 @default.
- W2045337251 cites W2130062777 @default.
- W2045337251 cites W2133727193 @default.
- W2045337251 cites W2140398365 @default.
- W2045337251 cites W2152101492 @default.
- W2045337251 cites W2155643523 @default.
- W2045337251 cites W2156754173 @default.
- W2045337251 cites W2157076525 @default.
- W2045337251 cites W2162633290 @default.
- W2045337251 cites W2164174765 @default.
- W2045337251 cites W2164886443 @default.
- W2045337251 cites W2166353621 @default.
- W2045337251 cites W2167912995 @default.
- W2045337251 cites W2168082049 @default.
- W2045337251 cites W2172292968 @default.
- W2045337251 cites W4253140965 @default.
- W2045337251 cites W52970530 @default.
- W2045337251 doi "https://doi.org/10.1016/j.nbd.2012.07.004" @default.
- W2045337251 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3992938" @default.
- W2045337251 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/22819776" @default.
- W2045337251 hasPublicationYear "2013" @default.
- W2045337251 type Work @default.
- W2045337251 sameAs 2045337251 @default.
- W2045337251 citedByCount "171" @default.
- W2045337251 countsByYear W20453372512012 @default.