Abnormal mitochondrial transport and morphology as early pathological changes in human models of spinal muscular atrophy

Spinal muscular atrophy (SMA), characterized by specific degeneration of spinal motor neurons, is caused by mutations in the survival of motor neuron 1, telomeric (SMN1) gene and subsequent decreased levels of functional SMN. How the deficiency of SMN, a ubiquitously expressed protein, leads to spin...

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Detalles Bibliográficos
Autores principales: Xu, Chong-Chong, Denton, Kyle R., Wang, Zhi-Bo, Zhang, Xiaoqing, Li, Xue-Jun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2016
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4728333/
https://www.ncbi.nlm.nih.gov/pubmed/26586529
http://dx.doi.org/10.1242/dmm.021766

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