Autophagy dysregulation in cell culture and animals models of spinal muscular atrophy

Abnormal autophagy has become a central thread linking neurodegenerative diseases, particularly of the motor neuron. One such disease is spinal muscular atrophy (SMA), a genetic neuromuscular disorder caused by mutations in the SMN1 gene resulting in low levels of Survival Motor Neuron (SMN) protein...

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Veröffentlicht in:	Molecular and cellular neuroscience 2014-07, Vol.61, p.133-140
Hauptverfasser:	Custer, Sara K., Androphy, Elliot J.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Autophagy Autophagy - physiology Cell Line, Transformed Disease Models, Animal Doxycycline - pharmacology Fibroblasts - drug effects Fibroblasts - metabolism Gene Expression Regulation - genetics Green Fluorescent Proteins - genetics Green Fluorescent Proteins - metabolism Luminescent Proteins - genetics Luminescent Proteins - metabolism Mice Microtubule-Associated Proteins - genetics Microtubule-Associated Proteins - metabolism Motor neuron Motor Neurons - physiology Muscular Atrophy, Spinal - genetics Muscular Atrophy, Spinal - pathology Muscular Atrophy, Spinal - physiopathology Red Fluorescent Protein RNA, Small Interfering - metabolism RNA, Small Interfering - pharmacology Spinal muscular atrophy Survival motor neuron Survival of Motor Neuron 1 Protein - genetics Survival of Motor Neuron 1 Protein - metabolism Time Factors Transfection
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