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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
Gene expression from human iPSC derived motor neurons.
Characterise the molecular pathogenesis of androgen receptor spinal and bulbar muscular atrop (SBMA) causing repeat expansion mutations in human motor neurons generated from patient-specific spinal cord motor neurons from induced pluripotent stem cells and performed time-resolved analysis of transcr...
ORGANISM(S): Homo sapiens 
Spinal Muscular Atrophy (SMA) is an autosomal recessive motor neuron disease and is the second most common genetic disorder leading to death in childhood. Motoneurons derived from induced pluripotent stem cells (iPSC) obtained by reprogramming SMA patient and his healthy father fibroblasts, and gene...
ORGANISM(S): Homo sapiens 
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ORGANISM(S): Homo sapiens 
We performed RNA-seq experiments on two samples (cortical neurons and spinal motor neurons) from normal induced pluripotent stem cells (iPSCs), and another two samples (cortical neurons and spinal motor neurons) derived from SPG3A (an early onset form of hereditary spastic paraplegia) iPSCs. This in...
ORGANISM(S): Homo sapiens 
Amyotrophic Lateral Sclerosis is a fatal neurodegenerative disorder that affects motor neurons (MN). We used single cell RNA-seq of degenerating human MN derived from ALS patients to understand molecular drivers of MN degeneration. Patient-derived iPSC bearing a point mutation in the SOD1 gene (SOD1...
ORGANISM(S): Homo sapiens 
Spinal Muscular Atrophy (SMA) is typically characterized as a motor neuron disease, but extra-neuronal phenotypes are present in almost every organ in severely affected patients and animal models. Extra-neuronal phenotypes were previously underappreciated as patients with severe SMA phenotypes usual...
ORGANISM(S): Homo Sapiens (human) 
Mutations in the AAA+ ATPase p97 cause multisystem proteinopathy 1 which includes amyotrophic-lateral sclerosis; however, the pathogenic mechanisms that contribute to motor neuron loss remain obscure. Here, we use two induced pluripotent stem cell models differentiated into spinal motor neurons to i...
ORGANISM(S): Mus musculus (Mouse) Homo sapiens (Human) 
2025-09-08 | PXD048185 | Pride
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