title: Vitamin D confers protection to motoneurons and is a prognostic factor of amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS) is an incurable paralytic disorder primarily typified by the selective and progressive degeneration of motoneurons in the brain and spinal cord. ALS causes muscle wasting and atrophy, resulting eventually in respiratory failure and death within 3-5 years of diagnosis. Vitamin D is a potent secosteroid hormone with diverse biological functions that include protection against neuronal damage. The detrimental consequences of vitamin D dietary deficiency have been documented in other neurodegenerative diseases. However, the protective effect of vitamin D on motoneuron and the influence of its levels on disease course remains elusive. Here we found that the biologically active form of vitamin D significantly potentiated the effect of neurotrophic factors and prevented motoneurons from a Fas-induced death, while electrophysiological properties of motoneurons were not affected. In ALS patients, we report that a severe vitamin D deficiency accelerates by 4 times the rate of decline and were associated with a marked shorter life expectancy. Our findings support a neuroprotective function of vitamin D on motoneurons and propose vitamin D as a reliable prognostic factor of ALS.
authors.affiliation: ALS Center, Department of Neurology, CHU Gui-de-Chauliac, University of Montpellier I, Montpellier, France; The Neuroscience Institute of Montpellier, Inserm UMR1051, Saint Eloi Hospital, Montpellier, France. Electronic address: w-camu@chu-montpellier.fr.
authors.forename: William
authors.initials: W
authors.lastname: Camu
authors.affiliation: The Neuroscience Institute of Montpellier, Inserm UMR1051, Saint Eloi Hospital, Montpellier, France.
authors.forename: Boris
authors.initials: B
authors.lastname: Tremblier
authors.affiliation: Laboratory of biostatistics, epidemiology and public health, University Institute of Clinical Research, University of Montpellier 1, Montpellier, France.
authors.forename: Carine
authors.initials: C
authors.lastname: Plassot
authors.affiliation: ALS Center, Department of Neurology, CHU Gui-de-Chauliac, University of Montpellier I, Montpellier, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Alphandery
authors.affiliation: The Neuroscience Institute of Montpellier, Inserm UMR1051, Saint Eloi Hospital, Montpellier, France.
authors.forename: Céline
authors.initials: C
authors.lastname: Salsac
authors.affiliation: ALS Center, Department of Neurology, CHU Gui-de-Chauliac, University of Montpellier I, Montpellier, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Pageot
authors.affiliation: ALS Center, Department of Neurology, CHU Gui-de-Chauliac, University of Montpellier I, Montpellier, France.
authors.forename: Raul
authors.initials: R
authors.lastname: Juntas-Morales
authors.affiliation: The Neuroscience Institute of Montpellier, Inserm UMR1051, Saint Eloi Hospital, Montpellier, France.
authors.forename: Frédérique
authors.initials: F
authors.lastname: Scamps
authors.affiliation: Laboratory of biostatistics, epidemiology and public health, University Institute of Clinical Research, University of Montpellier 1, Montpellier, France.
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Daures
authors.affiliation: The Neuroscience Institute of Montpellier, Inserm UMR1051, Saint Eloi Hospital, Montpellier, France.
authors.forename: Cédric
authors.initials: C
authors.lastname: Raoul
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D000818:Animals; D002470:Cell Survival / Q000187:drug effects*; D002478:Cells, Cultured; D053222:Fas Ligand Protein / Q000502:physiology; D005260:Female; D051100:Glial Cell Line-Derived Neurotrophic Factor / Q000494:pharmacology; D006801:Humans; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008875:Middle Aged; D009046:Motor Neurons / Q000187:drug effects*; D016501:Neurites / Q000502:physiology; D018696:Neuroprotective Agents; D015996:Survival Rate; D013997:Time Factors; D014807:Vitamin D / Q000008:administration & dosage / Q000494:pharmacology*
chemical_list: D053222:Fas Ligand Protein; D051100:Glial Cell Line-Derived Neurotrophic Factor; D018696:Neuroprotective Agents; D014807:Vitamin D
keywords: Fas; Motoneurons; Neurotrophic factor; Prognostic factor; Vitamin D