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MetabolomicsElevated brain manganese induces motor disease by upregulating the kynurenine pathway of tryptophan metabolism
Elevated brain levels of the essential metals manganese (Mn), copper, or iron induce motor disease. However, mechanisms of metal-induced motor disease are unclear and treatments are lacking. Elucidating the mechanisms of Mn-induced motor disease is particularly important because occupational and env...
2025-03-31 | MTBLS975 | MetaboLights
GenomicsSTAT3 promotes motor neuron differentiation by collaborating with motor neuron-specific LIM complex
The motor neuron (MN)–hexamer complex consisting of LIM homeobox 3, Islet-1, and nuclear LIM interactor is a key determinant of motor neuron specification and differentiation. To gain insights into the transcriptional network in motor neuron development, we performed a genome-wide ChIP-sequencing an...
ORGANISM(S): Mus musculus 
2013-09-19 | E-GEOD-50993 | biostudies-arrayexpress
TranscriptomicsAccelerated high-yield generation of limb-innervating motor neurons from human stem cells
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 
2013-02-06 | E-GEOD-41795 | biostudies-arrayexpress
TranscriptomicsPathways Disrupted in Human ALS Motor Neurons Identified Through Genetic Correction of Mutant SOD1
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 
2014-02-03 | E-GEOD-54409 | biostudies-arrayexpress
TranscriptomicsTranscriptome profiling of severe spinal muscular atrophy mouse embryonic stem cell-derived motor neurons
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 
2014-09-08 | E-GEOD-56284 | biostudies-arrayexpress
TranscriptomicsAn intricate interplay between astrocytes and motor neurons in ALS
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 
2013-02-11 | E-GEOD-43879 | biostudies-arrayexpress
TranscriptomicsTranscriptomic profiling of spinal and bulbar muscular atrophy cultured embryonic motor neurons
Spinal and bulbar muscular atrophy (SBMA), also known as Kennedy’s Disease, is a slowly progressive adult-onset neuromuscular disease which results from a polyglutamine (polyQ) encoding CAG repeat expansion within the androgen receptor gene (AR). Despite the ubiquitous expression of the androgen rec...
ORGANISM(S): Mus musculus 
2019-05-02 | E-MTAB-6986 | biostudies-arrayexpress
TranscriptomicsWhole genome transcriptome analysis identifies indices of fast and slow disease progression in two ALS mouse models
Microarray analysis has been applied to the study of ALS in order to investigate gene expression in whole spinal cord homogenates of SOD1 G93A mice and human ALS cases, although the massive presence of glial cells and inflammatory factors has made it difficult to define which gene expression changes...
ORGANISM(S): Mus musculus 
2013-10-01 | E-GEOD-46298 | biostudies-arrayexpress
TranscriptomicsiPSC derived motor neuron cultures from C9ORF72 carriers
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 
2013-11-07 | E-GEOD-52202 | biostudies-arrayexpress
ProteomicsAnalyzing the ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targets
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
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