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Axon-Specific Mitochondrial Pathology in SPG11 Alpha Motor Neurons

Pathogenic variants in SPG11 are the most frequent cause of autosomal recessive complicated hereditary spastic paraplegia (HSP). In addition to spastic paraplegia caused by corticospinal degeneration, most patients are significantly affected by progressive weakness and muscle wasting due to alpha mo...

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Автори: Fabian Güner, Tatyana Pozner, Florian Krach, Iryna Prots, Sandra Loskarn, Ursula Schlötzer-Schrehardt, Jürgen Winkler, Beate Winner, Martin Regensburger
Формат: Artigo
Мова:Inglês
Опубліковано: Frontiers Media S.A. 2021-07-01
Серія:Frontiers in Neuroscience
Предмети:
SPG11
hereditary spastic paraplegia
alpha motor neuron
induced pluripotent stem cells
mitochondria
axonal transport
Онлайн доступ:https://www.frontiersin.org/articles/10.3389/fnins.2021.680572/full
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