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Gene expression analysis of different motor neuron genotypes and their response to biological exposure and AAV treatment [neuropathology]

ABSTRACT: Wild-type motor neurons and motor neurons carrying ALS-relevant mutations (TDP-43 - M337V; SOD1 - G93A) were cultured and exposed different biological treatments in the absence and presence of AAV(s) (1-5) transduction and consequent protein expression. Their response to these variables under different combinations was assessed with regards to gene expression using nCounter Gene Expression Panels (nanoString), Neuropathology and Neuroinflammation Panels.

ORGANISM(S): Homo sapiens

PROVIDER: GSE281714 | GEO | 2024/12/01

REPOSITORIES: GEO

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Gene expression analysis of different motor neuron genotypes and their response to biological exposure and AAV treatment

Project description:Wild-type motor neurons and motor neurons carrying ALS-relevant mutations (TDP-43 - M337V; SOD1 - G93A) were cultured and exposed different biological treatments in the absence and presence of AAV(s) (1-5) transduction and consequent protein expression. Their response to these variables under different combinations was assessed with regards to gene expression using nCounter Gene Expression Panels (nanoString), Neuropathology and Neuroinflammation Panels.

2024-12-01 | GSE281698 | GEO

RNA-Seq result of Bulk spinal cord of SOD1*G93A transgenic mice treated with PF-04457845 or vehicle

Project description:SOD1*G93A transgenic mice were treated with PF-04457845 (a FAAH inhibitor)or vehicle . PF-04457845 extended survival of SOD1*G93A transgenic mice and resucued loss of motor neurons.

2023-09-18 | GSE242940 | GEO

Single cell RNA-Seq result of spinal cord of SOD1*G93A transgenic mice treated with PF-04457845 or vehicle

2023-09-18 | GSE242939 | GEO

Transcription profiling of mouse laser capture microdissected SOD1 G93A motor neurons reveals cellular pathways involved in the adaptation and progression of motor neuron injury in the mouse model of familial ALS

Project description: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. The aim of the present study is to combine LCM and microarray analysis to study how motor neurons in the spinal cord of transgenic SOD1 G93A mice and transgenic SOD1 WT respond to stimuli determined by the presence of the human mutant protein throughout the evolution of the stages in motor neuron injury Experiment Overall Design: Motor neurons have been isolated from the spinal cord of G93A mice and non transgenic littermates at different time points and the transcription expression profile of the isolated motor neurons has been analysed

2008-06-11 | E-GEOD-10953 | biostudies-arrayexpress

Cellular pathways involved in the adaptation and progression of motor neuron injury in the mouse model of familial ALS

2008-03-27 | GSE10953 | GEO

Modulation of histone H3K4 dimethylation by spermidine ameliorates motor neuron survival and neuropathology in a mouse model of ALS

Project description:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive paralysis due to motor neuron degeneration. It has been proposed that epigenetic modification and transcriptional dysregulation may contribute to motor neuron death. In this study, we investigate the basis for therapeutic approaches to target lysine-specific histone demethylase 1 (LSD1) and elucidate the mechanistic role of LSD1-histone H3K4 signaling pathway in ALS pathogenesis. Cellular and animal models of ALS were treated by spermidine (SD), then we performed neuropathological, body weight and survival evaluation in order to examine the role of SD in mutant superoxide dismutase 1 (mSOD1) (G93A) ALS mice. Herein, we found that dimethylated histone H3K4 (H3K4me2) levels are decreased in cellular and animal models of ALS, and correlated with motor neuronal dysfunction in ALS (G93A) mice. SD administration modulated the LSD1 activity and restored H3K4me2 levels in ChAT-positive motor neurons in the lumbar spinal cord of ALS mice. SD prevented cellular damage by improving the number and size of motor neurons in ALS mice. SD administration also reduced GFAP-positive astrogliogenesis in the white and gray matter of the lumbar spinal cord, improving the neuropathology of ALS mice. Moreover, SD administration delayed disease onset and prolonged the lifespan of ALS (G93A) transgenic mice. Together, modulating epigenetic targets such as LSD1 by small compounds may be a useful therapeutic strategy for treating ALS.

2022-09-13 | GSE213091 | GEO

miRNA expression in fast and slow skeletal muscle of wild type and SOD1-G93A mice

Project description:Amyotrophic lateral sclerosis (ALS) is a lethal motor neuron disease that progressively debilitates neuronal cells that control voluntary muscle activity. In a mouse model of ALS that expresses mutated human superoxide dismutase 1 (SOD1-G93A) skeletal muscle is one of the tissues affected early by mutant SOD1 toxicity. Fast-twitch and slow-twitch muscles are differentially affected in ALS patients and in the SOD1-G93A model, fast-twitch muscles being more vulnerable. We used miRNA microarrays to investigate miRNA alterations in fast-twitch (EDL) and slow-twitch (soleus) skeletal muscles of symptomatic SOD1-G93A animals and their age-matched wild type littermates.

2014-03-04 | GSE55507 | GEO

miRNA expression in fast and slow skeletal muscle of wild type and SOD1-G93A mice

2014-03-04 | E-GEOD-55507 | biostudies-arrayexpress

MiR-129-5p downregulates HuD expression impairing neuritogenesis in Amyotrophic Lateral Sclerosis

Project description:Amyotrophic lateral sclerosis (ALS) involves the degeneration of brain and spinal cord motor neurons. Mutations in Superoxide Dismutase 1 (SOD1), TAR DNA-binding protein 43 (TDP-43) and Fused-in-Sarcoma (FUS) account for 20-30 % of the familial ALS (fALS) cases. The RNA-binding proteins TDP-43 and FUS function in mRNA and miRNA biogenesis. MiRNAs are required for survival of neurons and deregulation of miRNA expression has been reported in several neurodegenerative disorders. Here, we report the dysregulation of DROSHA, DGCR8, and DICER in human neuroblastoma SH-SY5Y cells expressing the ALS-associated SOD1(G93A) mutant protein. MiRNA profiling in SH-SY5Y/SOD1(G93A) cells and transgenic SOD1(G93A) mice revealed upregulation of miR-129-5p at the early stage of disease. Moreover, miR-129-5p is also upregulated in lymphocytes of sporadic ALS patients. We demonstrate that miR-129-5p targets ELAVL4/HuD mRNA by binding to its 3’ UTR, which reduces HuD expression and impairs differentiation and neurite outgrowth. Conversely, treatment with an antagomir or complementation with HuD protein restores neuritogenesis. Collectively, our study identifies miR-129-5p and HuD as key regulators of neuronal differentiation and as potential therapeutic targets for ALS.

2018-02-01 | GSE94379 | GEO

Brown Adipose Tissue undergoes pathological perturbations and shapes C2C12 myoblast homeostasis in the SOD1-G93A mouse model of Amyotrophic Lateral Sclerosis.

Project description:Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the selective loss of motor neurons. While the contribution of peripheral organs remains incompletely understood, recent evidence suggests that brown adipose tissue (BAT) and its secreted extracellular vesicles (EVs) could play a role in diseased context as ALS. In this study, we employed a multi-omics approach, including RNA sequencing and proteomics, to investigate the alterations in BAT and its EVs in the SOD1-G93A mouse model of ALS. Our results revealed significant changes in the proteomic and transcriptomic profiles of BAT from SOD1-G93A mice, highlighting ALS-related features such as mitochondrial dysfunction and impaired differentiation capacity. Specifically, primary brown adipocytes (PBAs) from SOD1-G93A mice exhibited differentiation impairment, respiratory defects, and alterations in mitochondrial dynamics. Furthermore, the BAT-derived EVs from SOD1-G93A mice displayed distinct changes in size distribution and cargo content, which negatively impacted the differentiation and homeostasis of C2C12 murine myoblasts, as well as induced atrophy in C2C12-derived myotubes. These findings suggest that BAT undergoes pathological perturbations in ALS, contributing to skeletal muscle degeneration through the secretion of dysfunctional EVs. This study provides novel insights into the role of BAT in ALS pathogenesis and highlights potential therapeutic targets for mitigating muscle wasting in ALS patients.

2024-08-05 | GSE273052 | GEO

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