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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
ProteomicsTDP-43-stratified single-cell proteomic profiling of postmortem human spinal motor neurons reveals protein dynamics in amyotrophic lateral sclerosis
Single cell dataset of human motor neurons laser-captured from postmortem ALS and control tissues (1) ALS Pilot dataset (2) TDP43 stratified dataset Summary: Unbiased proteomics has been employed to interrogate central nervous system (CNS) tissues (brain, spinal cord) and fluid matrices (CSF, plas...
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2023-06-07 | MSV000092119 | MassIVE
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
TranscriptomicsGene expression in cellular compartments of Aplysia motor neurons
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 
2013-04-26 | E-GEOD-30387 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of human motor cortex in sporadic amyotrophic lateral sclerosis
We used oligonucleotide microarrays to find differentially expressed genes between control subjects and those affected by sporadic amyotrophic lateral sclerosis. Experiment Overall Design: Complementary RNAs (cRNAs) labelled with Cy5-CTP (Perkin-Elmer) were synthesized from 1 µg of total RNA of eac...
ORGANISM(S): Homo sapiens 
2008-06-13 | E-GEOD-4595 | 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
ProteomicsKIF1A Motor-Neck domain interaction
Quantitative Analysis of Motor-Neck Interaction by the Combination of Protein Painting and Stable Isotope Tags Labelling
ORGANISM(S): Sus Scrofa 
2021-12-23 | PXD026083 | JPOST Repository
ProteomicsHDAC6 inhibition restores TDP-43 pathology and axonal transport defects in human motor neurons with TARDBP mutations
TDP-43 is the major component of pathological inclusions in most ALS patients and in up to 50% of patients with frontotemporal dementia (FTD). Heterozygous missense mutations in TARDBP, the gene encoding TDP-43, are one of the common causes of familial ALS. In this study, we investigate TDP-43 prote...
ORGANISM(S): Homo sapiens (Human) 
2021-03-15 | PXD023852 | Pride
Induced pluripotent stem cells Axonal transport Tdp-43 Als Tardpb mutation Hdac6 Interactome 
ProteomicsLiver Steatosis and Mitochondrial Dysfunction in Spinal Muscular Atrophy is Survival Motor Neuron-Dependent and Hepatocyte-Intrinsic
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) 
2024-05-09 | PXD045401 | JPOST Repository
ProteomicsRat motor and sensory nerve LC-MSMS
Peripheral nerve repair and functional recovery depend on the rate of nerve regeneration and the quality of target reinnervation. It is important to fully understand the cellular and molecular basis underlying the specificity of peripheral nerve regeneration, which means the achieving of respective ...
ORGANISM(S): Rattus norvegicus (Rat) 
2020-12-10 | PXD022097 | Pride
Lc-msms Sensory nerve Rat Motor nerve Peripheral nerve injury 
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