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

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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Bibliographic Details
Main Authors: Chong-Chong Xu, Kyle R. Denton, Zhi-Bo Wang, Xiaoqing Zhang, Xue-Jun Li
Format: Article
Language:English
Published: The Company of Biologists 2016-01-01
Series:Disease Models & Mechanisms
Subjects:
Induced pluripotent stem cells
iPSCs
Human embryonic stem cells
hESCs
Spinal muscular atrophy
Mitochondrial transport and morphology
Online Access:http://dmm.biologists.org/content/9/1/39

Internet

http://dmm.biologists.org/content/9/1/39

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