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Human pluripotent stem cells are a promising source of diverse cells for developmental studies, cell transplantation, disease modeling, and drug testing. However, their widespread use even for intensely studied cell types like spinal motor neurons, is hindered by the long duration and low yields of...
ORGANISM(S): Homo sapiens 
Although many distinct mutations in a variety of genes are known to cause Amyotrophic Lateral Sclerosis (ALS), it remains poorly understood how they selectively impact motor neuron biology and whether they converge on common pathways to cause neural degeneration. Here, we have combined reprogramming...
ORGANISM(S): Homo sapiens 
Proximal spinal muscular atrophy (SMA) is an early onset, autosomal recessive motor neuron disease caused by loss of or mutation in SMN1 (survival motor neuron 1). Despite understanding the genetic basis underlying this disease, it is still not known why motor neurons (MNs) are selectively affected ...
ORGANISM(S): Mus musculus 
Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative condition characterized by loss of motor neurons in the brain and spinal cord. Expansions of a hexanucleotide repeat (GGGGCC) in the noncoding region of the C9ORF72 gene are the most common cause of the familial form of ALS (C9-ALS), ...
ORGANISM(S): Homo sapiens 
Supporting MS data files for paper (doi:10.3389/fncel.2023.1327361) by Watts M.E. et al., titled "Analyzing the ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targets". See attached pdf for index of MS files uploaded.
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2023-10-25 | MSV000093190 | MassIVE
Background: Differential gene expression specifies the highly diverse cell types that constitute the nervous system. With its sequenced genome and simple, well-defined neuroanatomy, the nematode C. elegans is a useful model system in which to correlate gene expression with neuron identity. The UNC-4...
ORGANISM(S): Caenorhabditis elegans 
Amyotrophic Lateral Sclerosis (ALS) results from the selective and progressive degeneration of motor neurons. Although the underlying disease mechanisms remain unknown, glial cells have been implicated in ALS disease progression. Here we examine the effects of glial cell/motor neuron interactions on...
ORGANISM(S): Mus musculus 
Whole genome transcriptional profiling is used to compare ESTs found in cell bodies and processes of Aplysia motor neurons RNA samples derived from cell bodies or processes of Aplysia single cultured motor neurons were hybridized to custom Aplysia EST microarrays. Two-condition experiment; four biol...
ORGANISM(S): Aplysia californica 
TDP-43, a DNA/RNA binding protein involved in RNA transcription and splicing has been associated with the pathophysiology of neurodegenerative diseases, including ALS. However, the function of TDP-43 in motor neurons remains undefined. Here, we employ both gain- and loss-of-function approaches to de...
ORGANISM(S): Mus musculus 
Spinal cord injury leads to impaired motor and sensory functions. After spinal cord injury there is a an initial phase of hypo-reflexia followed by a developing hyper-reflexia, often termed spasticity. Previous studies have suggested a relationship between the reappearence of plateau potentials in m...
ORGANISM(S): Rattus norvegicus 
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