Adherent Monomer-Misfolded SOD1
نویسندگان
چکیده
منابع مشابه
Adherent Monomer-Misfolded SOD1
BACKGROUND Multiple cellular functions are compromised in amyotrophic lateral sclerosis (ALS). In familial ALS (FALS) with Cu/Zn superoxide dismutase (SOD1) mutations, the mechanisms by which the mutation in SOD1 leads to such a wide range of abnormalities remains elusive. METHODOLOGY/PRINCIPAL FINDINGS To investigate underlying cellular conditions caused by the SOD1 mutation, we explored mut...
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Mice expressing variants of superoxide dismutase-1 (SOD1) encoding C-terminal truncation mutations linked to familial amyotrophic lateral sclerosis (FALS) have begun to define the role of misfolding and aggregation in the pathogenesis of disease. Here, we examine transgenic mice expressing SOD1-L126Z (Z = stop-truncation of last 28 amino acids), finding that detergent-insoluble mutant protein s...
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Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by loss of motor neurons. With conformation-specific antibodies, we now demonstrate that misfolded mutant SOD1 binds directly to the voltage-dependent anion channel (VDAC1), an integral membrane protein imbedded in the outer mitochondrial membrane. This interaction is fo...
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ژورنال
عنوان ژورنال: PLoS ONE
سال: 2008
ISSN: 1932-6203
DOI: 10.1371/journal.pone.0003497