GEO DataSets

(Submitter supplied) 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 were motor neuron specific. Recently, laser capture microdissection (LCM), combined with microarray analysis, has allowed the identification of motor neuron specific changes in gene expression in mouse and human ALS cases. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Platform:

GPL1261

64 Samples

Download data: CEL

(Submitter supplied) 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 were motor neuron specific. Recently, laser capture microdissection (LCM), combined with microarray analysis, has allowed the identification of motor neuron specific changes in gene expression in human ALS cases. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS3408

Platform:

GPL339

18 Samples

Download data: CEL

Analysis of motor neurons of 60, 90, and 120 day old SOD1 G93A transgenics, a model for familial amyotrophic lateral sclerosis (ALS). ALS is an adult-onset neurodegenerative disease characterized by degeneration of upper and lower motor neurons. Results provide insight into the pathogenesis of ALS.

Organism:

Mus musculus

Type:

Expression profiling by array, count, 3 age, 2 strain sets

Platform:

GPL339

Series:

GSE10953

18 Samples

Download data: CEL

(Submitter supplied) Burgeoning evidence highlights seminal roles for microglia in the pathogenesis of neurodegenerative diseases including amyotrophic lateral sclerosis (ALS). The receptor for advanced glycation end products (RAGE) binds ligands relevant to ALS that accumulate in the diseased spinal cord and RAGE has been previously implicated in the progression of ALS pathology. We generated a novel mouse model to temporally delete Ager from microglia in the murine SOD1G93A model of ALS. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24247

8 Samples

Download data: TXT

(Submitter supplied) To investigate the role of motor neuron autophagy in ALS, we generated mice in which the critical autophagy gene Atg7 was specifically disrupted in motor neurons (Atg7 cKO). We also bred these mice to the SOD1G93A mouse model of ALS. Then we performed RNA sequencing on lumbar spinal cords from these mice to determine how motor neuron autophagy inhibition altered gene expression.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL13112

16 Samples

Download data: CSV, TXT

(Submitter supplied) Missense mutations in the gene for the ubiquitously expressed superoxide dismutase-1 (SOD1) are one of the causes of familial amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disease in humans killing selectively large motor neurons. Mice and rats overexpressing mutant SOD1 develop an adult onset neurodegenerative disease with hindlimb-paralysis and subsequent death similar to the human condition. more...

Organism:

Rattus norvegicus

Type:

Expression profiling by array

Dataset:

GDS2823

Platform:

GPL1355

9 Samples

Download data: CEL

Analysis of spinal cord motor neurons from embryonic day 14 transgenics expressing the human superoxide dismutase 1 (SOD1) G93A mutant protein. The SOD1-G93A transgenic is a model for amyotrophic lateral sclerosis (ALS). Results provide insight into the pathogenesis of ALS.

Organism:

Rattus norvegicus

Type:

Expression profiling by array, count, 2 genotype/variation sets

Platform:

GPL1355

Series:

GSE7493

9 Samples

Download data: CEL

(Submitter supplied) In familial forms of amyotrophic lateral sclerosis (ALS) caused by mutations in superoxide dismutase-1 (SOD1) gene, both cell-autonomous and non-cell-autonomous mechanisms lead to the selective degeneration of motoneurons. Gene-targeted deletion of mutated SOD1 in mature astrocytes has been shown to slow down disease progression. However, the potential therapeutic application of targeting astrocytes has not been evaluated yet. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21103

13 Samples

Download data: TXT

(Submitter supplied) Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease in which motor neurons throughout the brain and spinal cord progressively degenerate resulting in muscle atrophy, paralysis and death. Recent studies using animal models of ALS implicate multiple cell-types (e.g., astrocytes and microglia) in ALS pathogenesis in the spinal motor systems. To ascertain cellular vulnerability and cell-type specific mechanisms of ALS in the brainstem that orchestrates oral-motor functions, we conducted parallel single cell RNA sequencing (scRNA-seq) analysis using the high-throughput Drop-seq method. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21103

4 Samples

Download data: TAR

(Submitter supplied) DNA sequence variants in the TBK1 gene associate with or cause sporadic or familial amyotrophic lateral sclerosis (ALS). Here we show that mice bearing human ALS-associated TBK1 missense loss-of-function mutations, or mice in which the Tbk1 gene is selectively deleted in motor neurons, do not display a neurodegenerative disease phenotype. However, loss of TBK1 function in motor neurons of the SOD1G93A mouse model of ALS impairs autophagy, increases SOD1 aggregation, and accelerates early disease onset without affecting lifespan. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21626

12 Samples

Download data: BW

(Submitter supplied) The role of glia in amyotrophic lateral sclerosis (ALS) is undeniable. Their disease-related activity has been extensively studied in the spinal cord, but only partly in the brain. We present herein a comprehensive study of glia in the motor cortex of SOD1(G93A) mice – a widely used model of ALS. Using single-cell RNA sequencing (scRNA-seq) and immunohistochemistry, we inspected astrocytes, microglia and oligodendrocytes, in four stages of the disease, respecting the factor of sex. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24247

8 Samples

Download data: CSV, TXT

(Submitter supplied) mRNA expression in the spinal cords of the G93A-SOD1 familial ALS transgenic mouse model was compared to that in nontransgenic (Normal mouse) and transgenic mice expressing wild-type (WT)SOD1. Gene Ontology (GO)analysis was used to characterize differences in expression between G93A-SOD1 mouse and nontransgenic mouse spinal cord. Changes in multiple GO categories were found. Many of these were associated with subsystems involving cell-cell communication and intracellular signal transduction. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS1747

Platform:

GPL81

6 Samples

Download data

Analysis of spinal cord from SOD1-G93A transgenic model of amyotrophic lateral sclerosis (ALS) at 75 or 110 days of age. Cu/Zn superoxide dismutase mutations are a cause of ALS. Gene expression results, together with proteomic data, provide insight into functional changes underlying ALS progression.

Organism:

Mus musculus

Type:

Expression profiling by array, count, 3 genotype/variation sets

Platform:

GPL81

Series:

GSE4390

6 Samples

Download data

(Submitter supplied) CNS-delivery of Interleukin 4 (IL-4) - via a lentiviral-mediated gene therapy strategy - skews microglia to proliferate, inducing these cells to adopt the phenotype of slowly proliferating cells. Transcriptome analysis revealed that IL-4-treated microglia express a broad number of genes normally encoded by embryonic microglia.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL17021

6 Samples

Download data: XLSX

(Submitter supplied) Gene expression profiling has been performed previously on motor cortex and spinal cord homogenates and of sporadic ALS cases and controls, to identify genes and pathways differentially expressed in ALS. More recent studies have combined the use of laser capture microdissection (LCM) with gene expression profiling to isolate the motor neurons from the surrounding cells, such as microglia and astrocytes, in order to determine those genes differentially expressed in the vulnerable cell population – i.e. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL570

10 Samples

Download data: CEL

(Submitter supplied) We report transcriptomic dysregulations in the spinal cord of asymptomatic (A: 1.5 months) symptomatic (S: 6 months) and end stage (E: 10-12 months) CHMP2Bintron5 mice by RNA sequencing. We found that genes related to immune system and lipid metabolism had altered expression levels at 1.5 month. Expression of genes related to neuronal system was atletred at 6months and expression of genes related to neuronal system and extracellular matrix xere dfound deregulated at the end-stage. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21103

32 Samples

Download data: TXT

(Submitter supplied) Translating ribosome affinity purification (TRAP) was performed on spinal cord dissections pooled from 3-4 mice 21 days post birth that were positive for the eGFP-L10A fusion ribosomal marker protein under the expression of either the Chat promoter (Tg(Chat-EGFP/Rpl10a)DW167Htz) or the Snap25 promoter (Tg(Snap25-EGFP/Rpl10a)JD362Jdd). RNA-sequencing was performed on both TRAP and pre-immunoprecipitation (PreIP) control RNA samples.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL17021

12 Samples

Download data: CSV

(Submitter supplied) Although ALS typically presents in mid to late-life, there is increasing evidence for a protracted preclinical period of motor neuron dysfunction. The goal of this study is to identify the earliest gene expression patterns in lower motor neurons that drive selective neuronal vulnerability in a mouse model of ALS. We have implemented transgenic SOD1G93A with a lower motor neuron fluorescent reporter (HB9-GFP) mice to unambiguously isolate spinal motor neurons using FACS for gene expression profiling using RNA sequencing at embryonic day (E)12.5. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL13112

8 Samples

Download data: CSV, TXT

(Submitter supplied) CRISPR-based gene editing technology represents a promising approach to deliver therapies for inherited disorders, including amyotrophic lateral sclerosis (ALS). Toxic gain-of-function superoxide dismutase 1 (SOD1) mutations are responsible for ~20% of familial ALS cases. Thus, current clinical strategies to treat SOD1-ALS are designed to lower SOD1 levels. Here, we utilized AAV-PHP.B variants to deliver CRISPR-Cas9 guide RNAs designed to disrupt the human SOD1 (huSOD1) transgene in SOD1G93A mice. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24676

40 Samples

Download data: TSV

(Submitter supplied) Microarray analysis of microglia in a mouse model of amyotrophic lateral sclerosis identified the dysregulation of Brca1.

Organism:

Mus musculus

Type:

Expression profiling by array

Platform:

GPL1261

6 Samples

Download data: CEL