Ultrastructural features of aberrant glial cells isolated from the spinal cord of paralytic rats expressing the amyotrophic lateral sclerosis-linked SOD1G93A mutation

2017 ◽  
Vol 370 (3) ◽  
pp. 391-401 ◽  
Author(s):  
Marcie Jiménez-Riani ◽  
Pablo Díaz-Amarilla ◽  
Eugenia Isasi ◽  
Gabriela Casanova ◽  
Luis Barbeito ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Glial Cells ◽  
Aberrant Glial Cells ◽  
Lateral Sclerosis

scholarly journals How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis

Cells ◽  
2020 ◽  
Vol 9 (12) ◽  
pp. 2550
Author(s):  
Roxane Crabé ◽  
Franck Aimond ◽  
Philippe Gosset ◽  
Frédérique Scamps ◽  
Cédric Raoul
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Glial Cells ◽  
Neurological Disorder ◽  
Cellular Network ◽  
Motor System ◽  
Progressive Degeneration ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by the progressive degeneration of upper and lower motoneurons. Despite motoneuron death being recognized as the cardinal event of the disease, the loss of glial cells and interneurons in the brain and spinal cord accompanies and even precedes motoneuron elimination. In this review, we provide striking evidence that the degeneration of astrocytes and oligodendrocytes, in addition to inhibitory and modulatory interneurons, disrupt the functionally coherent environment of motoneurons. We discuss the extent to which the degeneration of glial cells and interneurons also contributes to the decline of the motor system. This pathogenic cellular network therefore represents a novel strategic field of therapeutic investigation.

scholarly journals Mitochondrial Modulation by Dichloroacetate Reduces Toxicity of Aberrant Glial Cells and Gliosis in the SOD1G93A Rat Model of Amyotrophic Lateral Sclerosis

2018 ◽  
Vol 16 (1) ◽  
pp. 203-215 ◽  
Author(s):  
Laura Martínez-Palma ◽  
Ernesto Miquel ◽  
Valentina Lagos-Rodríguez ◽  
Luis Barbeito ◽  
Adriana Cassina ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Glial Cells ◽  
Rat Model ◽  
Aberrant Glial Cells ◽  
Lateral Sclerosis

scholarly journals A 14-3-3 mRNA Is Up-Regulated in Amyotrophic Lateral Sclerosis Spinal Cord

2008 ◽  
Vol 75 (6) ◽  
pp. 2511-2520 ◽  
Author(s):  
Andrea Malaspina ◽  
Narendra Kaushik ◽  
Jackie de Belleroche
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Lateral Sclerosis

scholarly journals RBM45 Modulates the Antioxidant Response in Amyotrophic Lateral Sclerosis through Interactions with KEAP1

2015 ◽  
Vol 35 (14) ◽  
pp. 2385-2399 ◽  
Author(s):  
Nadine Bakkar ◽  
Arianna Kousari ◽  
Tina Kovalik ◽  
Yang Li ◽  
Robert Bowser
Keyword(s):  
Oxidative Stress ◽  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Rna Binding ◽  
Binding Protein ◽  
Rna Binding Protein ◽  
Antioxidant Response ◽  
Cytoplasmic Inclusions ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the selective loss of motor neurons. Various factors contribute to the disease, including RNA binding protein dysregulation and oxidative stress, but their exact role in pathogenic mechanisms remains unclear. We have recently linked another RNA binding protein, RBM45, to ALS via increased levels of protein in the cerebrospinal fluid of ALS patients and its localization to cytoplasmic inclusions in ALS motor neurons. Here we show RBM45 nuclear exit in ALS spinal cord motor neurons compared to controls, a phenotype recapitulatedin vitroin motor neurons treated with oxidative stressors. We find that RBM45 binds and stabilizes KEAP1, the inhibitor of the antioxidant response transcription factor NRF2. ALS lumbar spinal cord lysates similarly show increased cytoplasmic binding of KEAP1 and RBM45. Binding of RBM45 to KEAP1 impedes the protective antioxidant response, thus contributing to oxidative stress-induced cellular toxicity. Our findings thus describe a novel link between a mislocalized RNA binding protein implicated in ALS (RBM45) and dysregulation of the neuroprotective antioxidant response seen in the disease.

scholarly journals Optineurin pathology in the spinal cord of amyotrophic lateral sclerosis/parkinsonism-dementia complex patients in Kii Peninsula, Japan

2018 ◽  
Vol 28 (3) ◽  
pp. 422-426 ◽  
Author(s):  
Satoru Morimoto ◽  
Hiroyuki Hatsuta ◽  
Rie Motoyama ◽  
Yasumasa Kokubo ◽  
Hiroyuki Ishiura ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Kii Peninsula ◽  
Complex Patients ◽  
Lateral Sclerosis
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