Absence of lipofuscin in motor neurons of SOD1-linked ALS mice
نویسندگان
چکیده
منابع مشابه
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.
The most common inherited [correct] form of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1). In chimeric mice that are mixtures of normal and SOD1 mutant-expressing cells, toxicity to motor neurons is shown to require damage from mutant SOD1 acting with...
متن کاملAggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1.
Analysis of transgenic mice expressing familial amyotrophic lateral sclerosis (ALS)-linked mutations in the enzyme superoxide dismutase (SOD1) have shown that motor neuron death arises from a mutant-mediated toxic property or properties. In testing the disease mechanism, both elimination and elevation of wild-type SOD1 were found to have no effect on mutant-mediated disease, which demonstrates ...
متن کاملToxicity of ALS-linked SOD1 mutants.
Estévez et al. (1) focused on a potential mechanism through which dominantly inherited mutation in superoxide dismutase 1 (SOD1), an abundant, ubiquitously expressed antioxidant protein, triggers the selective death of motor neurons in amyotrophic lateral sclerosis (ALS). Each subunit of SOD1 binds one zinc and one copper atom. Dismutation of the superoxide radical to H2O2 or O2 requires enzyme...
متن کاملExcitation BolsTORs Motor Neurons in ALS Mice
It is unclear why motor neurons selectively degenerate in amyotrophic lateral sclerosis (ALS). Saxena et al. (2013) demonstrate that excitation of motor neurons can prevent their demise in a mouse model of inherited ALS by a mechanism involving the mTOR pathway.
متن کاملMutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice.
Dominant mutations in ubiquitously expressed superoxide dismutase (SOD1) cause familial ALS by provoking premature death of adult motor neurons. To test whether mutant damage to cell types beyond motor neurons is required for the onset of motor neuron disease, we generated chimeric mice in which all motor neurons and oligodendrocytes expressed mutant SOD1 at a level sufficient to cause fatal, e...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2014
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.1409314111