| http://purl.uniprot.org/citations/14962977 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/14962977 | http://www.w3.org/2000/01/rdf-schema#comment | "Extensive striatal neuronal loss occurs in Huntington's disease (HD), which is caused by an expanded polyglutamine tract in huntingtin (htt). Evidence suggests that mutant htt directly or indirectly compromises mitochondrial function, contributing to the neuronal loss. To determine the role of compromised mitochondrial function in the neuronal cell death in HD, immortalized striatal cells established from Hdh(Q7) (wild-type) and Hdh(Q111) (mutant) mouse knock-in embryos were treated with 3-nitropropionic acid (3-NP), a mitochondrial complex II toxin. 3-NP treatment caused significantly greater cell death in mutant striatal cells compared with wild-type cells. In contrast, the extent of cell death induced by rotenone, a complex I inhibitor, was similar in both cell lines. Although evidence of apoptosis was present in 3-NP-treated wild-type striatal cells, it was absent in 3-NP-treated mutant cells. 3-NP treatment caused a greater loss of mitochondrial membrane potential (deltapsim) in mutant striatal cells compared with wild-type cells. Cyclosporine A, an inhibitor of mitochondrial permeability transition pore (PTP), and ruthenium red, an inhibitor of the mitochondrial calcium uniporter, both rescued mutant striatal cells from 3-NP-induced cell death and prevented the loss of deltapsim. These data show that mutant htt specifically increases cell vulnerability to mitochondrial complex II inhibition and further switched the type of cell death induced by complex II inhibition from apoptosis to a non-apoptotic form, caused by mitochondrial membrane depolarization, probably initiated by mitochondrial calcium overload and subsequent PTP opening. These findings suggest that impaired mitochondrial complex II function in HD may contribute to non-apoptotic neuronal cell death."xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.org/dc/terms/identifier | "doi:10.1093/hmg/ddh082"xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/author | "MacDonald M.E."xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/author | "Ruan Q."xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/author | "Johnson G.V."xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/author | "Lesort M."xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/date | "2004"xsd:gYear |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/name | "Hum Mol Genet"xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/pages | "669-681"xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/title | "Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway."xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://purl.uniprot.org/core/volume | "13"xsd:string |
| http://purl.uniprot.org/citations/14962977 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/14962977 |
| http://purl.uniprot.org/citations/14962977 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/14962977 |
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| http://purl.uniprot.org/uniprot/#_G3X9H5-mappedCitation-14962977 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/14962977 |
| http://purl.uniprot.org/uniprot/P42859 | http://purl.uniprot.org/core/mappedCitation | http://purl.uniprot.org/citations/14962977 |
| http://purl.uniprot.org/uniprot/G3X9H5 | http://purl.uniprot.org/core/mappedCitation | http://purl.uniprot.org/citations/14962977 |