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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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Principais autores: Fabian Güner, Tatyana Pozner, Florian Krach, Iryna Prots, Sandra Loskarn, Ursula Schlötzer-Schrehardt, Jürgen Winkler, Beate Winner, Martin Regensburger
Formato: Artigo
Idioma:Inglês
Publicado em: Frontiers Media S.A. 2021-07-01
coleção:Frontiers in Neuroscience
Assuntos:
SPG11
hereditary spastic paraplegia
alpha motor neuron
induced pluripotent stem cells
mitochondria
axonal transport
Acesso em linha:https://www.frontiersin.org/articles/10.3389/fnins.2021.680572/full
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