Granulocyte-colony stimulating factor (G-CSF) has been recently identified as a neurotrophic factor able to preserve motor functions, rescue motor units and extent survival in an animal model of amyotrophic lateral sclerosis, the SOD1 G93A mice. To gain insight into the mode of action of G-CSF, we have recently performed gene expression profiling on isolated lumbar motoneurons from SOD1G93A mice, and shown that G-CSF re-adjusted gene expression in motoneurons of symptomatic SOD1G93A mice and modulates genes related to neuromuscular function (Henriques et al., 2015). Here, we provide quality controls for the microarray experiment (GO accession number GSE60856) and describe the experimental strategy.
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http://dx.doi.org/10.1016/j.gdata.2015.02.003 | DOI Listing |
Ann Neurol
January 2025
Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA.
Int J Mol Sci
November 2024
Neurocentre Magendie INSERM U1215, Université de Bordeaux, 33000 Bordeaux, France.
In amyotrophic lateral sclerosis (ALS), early mitochondrial dysfunction may contribute to progressive motor neuron loss. Remarkably, the ectopic expression of the Orthobornavirus bornaense type 1 (BoDV-1) X protein in mitochondria blocks apoptosis and protects neurons from degeneration. Therefore, this study examines the neuroprotective effects of X protein in an ALS mouse model.
View Article and Find Full Text PDFResearch (Wash D C)
December 2024
Department of Medical Genetics and Center for Rare Diseases, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Disease-associated microglia (DAM) are observed in neurodegenerative diseases, demyelinating disorders, and aging. However, the spatiotemporal dynamics and evolutionary trajectory of DAM during the progression of amyotrophic lateral sclerosis (ALS) remain unclear. Using a mouse model of ALS that expresses a human gene mutation, we found that the microglia subtype DAM begins to appear following motor neuron degeneration, primarily in the brain stem and spinal cord.
View Article and Find Full Text PDFJ Control Release
December 2024
School of Chemistry and Molecular Bioscience, Molecular Horizons, Faculty of Science, Medicine and Health, University of Wollongong, Wollongong, NSW 2522, Australia. Electronic address:
Front Neurosci
November 2024
Aging and Metabolism Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, United States.
Our study investigated the therapeutic potential of OKN-007 in the SOD1 G93A mouse model of amyotrophic lateral sclerosis (ALS). The impact of OKN-007, known for its antioxidant, anti-inflammatory, and neuroprotective properties, was tested at two doses (150 mg/kg and 300 mg/kg) at onset and late-stage disease. Results demonstrated a significant delay in disease progression at both doses, with treated mice showing a slower advance to early disease stages compared to untreated controls.
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