title: Methylcobalamin prevents mutant superoxide dismutase-1-induced motor neuron death in vitro.
abstract: Amyotrophic lateral sclerosis (ALS) is an incurable progressive neurodegenerative disorder that causes motor dysfunction. Treatments and drugs that slow progression of ALS have garnered great interest. In the present study, we show that the vitamin B12 analog methylcobalamin (MBL) effectively and dose dependently prevented embryonic stem cell-derived motor neuron death induced by cocultivation with astrocytes expressing mutant human superoxide dismutase-1 (G93A). Moreover, cotreatment of MBL with a conventional ALS drug, riluzole, further enhanced survival of motor neurons in this in-vitro ALS model. Our results show the potential use of MBL as a treatment for ALS and suggest a possible combination therapy strategy with other types of approved ALS drugs.
authors.affiliation: Departments of aDevelopmental Neurobiology bClinical Science, KAN Research Institute Inc., Kobe, Hyogo Prefecture, Japan cNeurology Business Group, Eisai Co. Ltd, Tsukuba, Ibaraki Prefecture, Japan.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Ito
authors.forename: Yukina
authors.initials: Y
authors.lastname: Izumi
authors.forename: Tetsuhiro
authors.initials: T
authors.lastname: Niidome
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Ono
mesh_terms: D000818:Animals; D016923:Cell Death / Q000187:drug effects* / Q000235:genetics; D002478:Cells, Cultured; D002540:Cerebral Cortex / Q000166:cytology; D004622:Embryo, Mammalian; D020287:In Situ Nick-End Labeling; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000187:drug effects* / Q000502:physiology*; D018696:Neuroprotective Agents / Q000494:pharmacology; D019782:Riluzole / Q000494:pharmacology; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism; D014805:Vitamin B 12 / Q000031:analogs & derivatives* / Q000494:pharmacology; D014803:Vitamin B Complex / Q000494:pharmacology*
chemical_list: D018696:Neuroprotective Agents; D014803:Vitamin B Complex; D019782:Riluzole; C019476:mecobalamin; C109415:SOD1 G93A protein; D013482:Superoxide Dismutase; D014805:Vitamin B 12