Mitochondrial respiratory chain deficiency correlates with the severity of neuropathology in sporadic Creutzfeldt-Jakob disease

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作者
Irene H. Flønes
Gerda Ricken
Sigrid Klotz
Alexandra Lang
Thomas Ströbel
Christian Dölle
Gabor G. Kovacs
Charalampos Tzoulis
机构
[1] Haukeland University Hospital,Neuro
[2] University of Bergen,SysMed, Department of Neurology
[3] Medical University of Vienna,Department of Clinical Medicine
[4] University of Toronto,Institute of Neurology
[5] University Health Network,Tanz Centre for Research in Neurodegenerative Disease
关键词
Mitochondria; Respiratory chain; Prion; Neurodegeneration; PrP;
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摘要
Mitochondrial dysfunction has been implicated in multiple neurodegenerative diseases but remains largely unexplored in Creutzfeldt-Jakob disease. Here, we characterize the mitochondrial respiratory chain at the individual neuron level in the MM1 and VV2 common molecular subtypes of sporadic Creutzfeldt-Jakob disease. Moreover, we investigate the associations between the mitochondrial respiratory chain and neuropathological markers of the disease.
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