Article
Preserved striatal innervation maintains motor function despite severe loss of nigral dopaminergic neurons.
Brain : a journal of neurology - 3 Sept 2024
Paß Thomas, Ricke Konrad M, Hofmann Pierre, Chowdhury Roy S, Nie Yu, Chinnery Patrick, Endepols Heike, Neumaier Bernd, Carvalho André, Rigoux Lionel, Steculorum Sophie M, Prudent Julien, Riemer Trine, Aswendt Markus, Liss Birgit, Brachvogel Bent, Wiesner Rudolf J
Abstract excerpt
Degeneration of dopaminergic neurons in the substantia nigra and their striatal axon terminals causes cardinal motor symptoms of Parkinson's disease. In idiopathic cases, high levels of mitochondrial DNA alterations, leading to mitochondrial dysfunction, are a central feature of these vulnerable neurons. Here we present a mouse model expressing the K320E variant of the mitochondrial helicase Twinkle in...
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