Mitochondrial Function, Morphology, and Axonal Transport in Amyotrophic Lateral Sclerosis
نویسندگان
چکیده
منابع مشابه
Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.
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 ...
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SOD1 is a cause of the fatal, paralytic disorder ALS. Although mechanisms underlying mutant SOD1 neurotoxicity remain uncertain, this protein associates with mitochondria. In this issue of Neuron, Israelson et al. show that mutant SOD1 binds and inhibits the mitochondrial channel VDAC1. This finding sheds light onto possible molecular links between mutant SOD1, mitochondrial dysfunction, and sp...
متن کاملAxonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis.
Axonal transport deficits have been reported in many neurodegenerative conditions and are widely assumed to be an immediate causative step of axon and synapse loss. By imaging changes in axonal morphology and organelle transport over time in several animal models of amyotrophic lateral sclerosis (ALS), we now find that deficits in axonal transport of organelles (mitochondria, endosomes) and axo...
متن کاملAxonal ion channel dysfunction in c9orf72 familial amyotrophic lateral sclerosis.
IMPORTANCE Abnormalities of axonal excitability characterized by upregulation of persistent sodium (Na+) conductances and reduced potassium (K+) currents have been reported in sporadic amyotrophic lateral sclerosis (SALS) phenotypes and linked to the development of clinical features such as fasciculations and neurodegeneration. OBJECTIVE To investigate whether abnormalities of axonal ion chan...
متن کاملComparing axonal excitability in past polio to amyotrophic lateral sclerosis.
INTRODUCTION Poliomyelitis causes selective destruction of anterior horn cells and usually has a stable disease course post-infection. We assessed the excitability characteristics in patients with a stable course after past poliomyelitis and compared them with changes described in amyotrophic lateral sclerosis (ALS). METHODS The excitability characteristics of motor and sensory nerves were st...
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ژورنال
عنوان ژورنال: Antioxidants & Redox Signaling
سال: 2009
ISSN: 1523-0864,1557-7716
DOI: 10.1089/ars.2009.2604