Sensory neurons do not induce motor neuron loss in a human stem cell model of spinal muscular atrophy. PLoS One 2014;9(7):e103112
Date
07/24/2014Pubmed ID
25054590Pubmed Central ID
PMC4108398DOI
10.1371/journal.pone.0103112Scopus ID
2-s2.0-84904700148 (requires institutional sign-in at Scopus site) 14 CitationsAbstract
Spinal muscular atrophy (SMA) is an autosomal recessive disorder leading to paralysis and early death due to reduced SMN protein. It is unclear why there is such a profound motor neuron loss, but recent evidence from fly and mouse studies indicate that cells comprising the whole sensory-motor circuit may contribute to motor neuron dysfunction and loss. Here, we used induced pluripotent stem cells derived from SMA patients to test whether sensory neurons directly contribute to motor neuron loss. We generated sensory neurons from SMA induced pluripotent stem cells and found no difference in neuron generation or survival, although there was a reduced calcium response to depolarizing stimuli. Using co-culture of SMA induced pluripotent stem cell derived sensory neurons with control induced pluripotent stem cell derived motor neurons, we found no significant reduction in motor neuron number or glutamate transporter boutons on motor neuron cell bodies or neurites. We conclude that SMA sensory neurons do not overtly contribute to motor neuron loss in this human stem cell system.
Author List
Schwab AJ, Ebert ADAuthor
Allison D. Ebert PhD Associate Professor in the Cell Biology, Neurobiology and Anatomy department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
CalciumCell Line
Cell Survival
Coculture Techniques
Humans
Motor Neurons
Muscular Atrophy, Spinal
Neurogenesis
Sensory Receptor Cells
Stem Cells