Article
Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.
Human molecular genetics - 15 Nov 2007
De Vos Kurt J, Chapman Anna L, Tennant Maria E, Manser Catherine, Tudor Elizabeth L, Lau Kwok-Fai, Brownlees Janet, Ackerley Steven, Shaw Pamela J, McLoughlin Declan M, Shaw Christopher E, Leigh P Nigel, Miller Christopher C J, Grierson Andrew J
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a late-onset neurological disorder characterized by death of motoneurons. Mutations in Cu/Zn superoxide dismutase-1 (SOD1) cause familial ALS but the mechanisms whereby they induce disease are not fully understood. Here, we use time-lapse microscopy to monitor for the first time the effect of mutant SOD1 on fast axonal transport (FAT) of bona fide cargoes in living neurons....
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