Combined form of Charcot disease and osteomalacia

Amyotrophic lateral sclerosis, described for the first time in 1869 - 1874 by Charcot and Joffrois and which received more accurate and complete coverage in the future thanks to the studies of Flсhsig, is known to be characterized by primary systemic degeneration along the cortical-muscular pathway, including here, in addition to the spinal cord department, and bulbar, related to the muscles of the face, tongue, soft palate, etc.; the entire conductive path is not always captured, but only a significant part of it

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Motor ekoked potentials to transcranial and spinal cord magnetic stimulation in amyotrophic lateral sclerosis (ALS)

Electroencephalography and Clinical Neurophysiology ◽

10.1016/s0013-4694(97)88792-9 ◽

1997 ◽

Vol 103 (1) ◽

pp. 169-170

Author(s):

A Ghezzi

Keyword(s):

Spinal Cord ◽

Amyotrophic Lateral Sclerosis ◽

Magnetic Stimulation ◽

Lateral Sclerosis

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Faculty Opinions recommendation of Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1022215.14073074 ◽

2011 ◽

Author(s):

Guy Rouleau

Keyword(s):

Spinal Cord ◽

Amyotrophic Lateral Sclerosis ◽

Mutant Proteins ◽

Sod1 Mutant ◽

Lateral Sclerosis

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

Journal of Neurochemistry ◽

10.1046/j.1471-4159.2000.0752511.x ◽

2008 ◽

Vol 75 (6) ◽

pp. 2511-2520 ◽

Cited By ~ 39

Author(s):

Andrea Malaspina ◽

Narendra Kaushik ◽

Jackie de Belleroche

Keyword(s):

Spinal Cord ◽

Amyotrophic Lateral Sclerosis ◽

Lateral Sclerosis

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

Molecular and Cellular Biology ◽

10.1128/mcb.00087-15 ◽

2015 ◽

Vol 35 (14) ◽

pp. 2385-2399 ◽

Cited By ~ 12

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.

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