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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
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
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
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
TranscriptomicsCLP1 links tRNA metabolism to progressive motor-neuron loss
CLP1 was the first mammalian RNA kinase to be identified. However, determining its in vivo function has been elusive. Here we generated kinase-dead Clp1 (Clp1K/K) mice that show a progressive loss of spinal motor neurons associated with axonal degeneration in the peripheral nerves and denervation of...
ORGANISM(S): Mus musculus 
2013-03-12 | E-GEOD-35924 | biostudies-arrayexpress
TranscriptomicsEffects of human skeletal muscle fibroblast and stem cell media on cultured rat motor neuron cells
The study was designed to compare whether and how muscle fibroblasts (FIB) and muscle stem cells (MYO) interact with motor neurons. Moreover, we aimed to investigate whether muscle cells, isolated from lifelong exercisers, exerted a protective and supportive effect on motor neurons. These object...
ORGANISM(S): Rattus norvegicus 
2026-02-21 | E-MTAB-14399 | 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
TranscriptomicsA SUMOylated C-terminus fragment of glutamate transporter EAAT2 linked to inherited ALS mediates motor axon impairment
Downregulation of expression and activity levels of the astroglial glutamate transporter EAAT2 is thought to be implicated in motor neuron excitotoxicity in amyotrophic lateral sclerosis (ALS). We previously reported that EAAT2 is cleaved by caspase-3 at the cytosolic C-terminus domain, impairing th...
ORGANISM(S): Mus musculus 
2011-07-19 | E-GEOD-26070 | biostudies-arrayexpress
TranscriptomicsToxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss
FUS is an RNA-binding protein involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Cytoplasmic FUS-containing aggregates are often associated with concomitant loss of nuclear FUS. Whether loss of nuclear FUS function, gain of a cytoplasmic function, or a combination of ...
ORGANISM(S): Mus musculus 
2016-03-14 | E-GEOD-78730 | biostudies-arrayexpress
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