Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form. - Wikidata - awgldk - TriplyDB

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form.

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form.

http://www.wikidata.org/entity/Q30156974

about

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis Tempol moderately extends survival in a hSOD1(G93A) ALS rat model by inhibiting neuronal cell loss, oxidative damage and levels of non-native hSOD1(G93A) forms The complex molecular biology of amyotrophic lateral sclerosis (ALS)Intermolecular transmission of superoxide dismutase 1 misfolding in living cells.Oligomerization of Cu,Zn-Superoxide Dismutase (SOD1) by Docosahexaenoic Acid and Its Hydroperoxides In Vitro: Aggregation Dependence on Fatty Acid Unsaturation and Thiols.Ultrafast colorimetric determination of predominant protein structure evolution with gold nanoplasmonic particles.Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.The impact of fraudulent and irreproducible data to the translational research crisis - solutions and implementation.Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants.Multiple Aggregation Pathways in Human γS-Crystallin and Its Aggregation-Prone G18V Variant.Nuclear export of misfolded SOD1 mediated by a normally buried NES-like sequence reduces proteotoxicity in the nucleus.Protease-resistant SOD1 aggregates in amyotrophic lateral sclerosis demonstrated by paraffin-embedded tissue (PET) blot.Motor neuron disease due to neuropathy target esterase gene mutation: clinical features of the index families.Cu, Zn-superoxide dismutase 1 (SOD1) is a novel target of Puromycin-sensitive aminopeptidase (PSA/NPEPPS): PSA/NPEPPS is a possible modifier of amyotrophic lateral sclerosis.Proteasome activation is a mechanism for pyrazolone small molecules displaying therapeutic potential in amyotrophic lateral sclerosis Nonamyloid aggregates arising from mature copper/zinc superoxide dismutases resemble those observed in amyotrophic lateral sclerosis Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes Decreased stability and increased formation of soluble aggregates by immature superoxide dismutase do not account for disease severity in ALS.Identification of human monoclonal antibodies specific for human SOD1 recognizing distinct epitopes and forms of SOD1.Cupric ions induce the oxidation and trigger the aggregation of human superoxide dismutase 1 Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis.Motor neuron disease due to neuropathy target esterase mutation: enzyme analysis of fibroblasts from human subjects yields insights into pathogenesis.Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis.SOD1 oxidation and formation of soluble aggregates in yeast: relevance to sporadic ALS development.Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.TDP-43 skeins show properties of amyloid in a subset of ALS cases.Morpholino-mediated SOD1 reduction ameliorates an amyotrophic lateral sclerosis disease phenotype.TDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashion ALS-linked misfolded SOD1 species have divergent impacts on mitochondria.Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis.Endogenous macrophage migration inhibitory factor reduces the accumulation and toxicity of misfolded SOD1 in a mouse model of ALS.Evidence for prion-like mechanisms in several neurodegenerative diseases: potential implications for immunotherapy Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALS TDP-43 inclusion bodies formed in bacteria are structurally amorphous, non-amyloid and inherently toxic to neuroblastoma cells.Immunochemical characterization on pathological oligomers of mutant Cu/Zn-superoxide dismutase in amyotrophic lateral sclerosis.Additive amelioration of ALS by co-targeting independent pathogenic mechanisms.Proteostasis and movement disorders: Parkinson's disease and amyotrophic lateral sclerosis.

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P2860

Amyotrophic lateral sclerosis is a non-amyloid disease in which extensive misfolding of SOD1 is unique to the familial form.

http://www.wikidata.org/entity/Q30156974

description

2010 nî lūn-bûn

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2010 թուականի Յունուարին հրատարակուած գիտական յօդուած

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2010 թվականի հունվարին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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Amyotrophic lateral sclerosis ...... s unique to the familial form.

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Amyotrophic lateral sclerosis ...... s unique to the familial form.

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Amyotrophic lateral sclerosis ...... s unique to the familial form.

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Amyotrophic lateral sclerosis ...... s unique to the familial form.

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Amyotrophic lateral sclerosis ...... s unique to the familial form.

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S00401-010-0646-5

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Acta Neuropathologica

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Aaron Kerman

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Avijit Chakrabartty

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Hsueh-Ning Liu

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Janice Robertson

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Juan Bilbao

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Lorne Zinman

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Sidney Croul

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P2860

Methods for staining amyloid in tissues: a review

Protein misfolding and the serpinopathies.

Conformational constraints for amyloid fibrillation: the importance of being unfolded.

Structure, folding, and misfolding of Cu,Zn superoxide dismutase in amyotrophic lateral sclerosis.

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s00401-010-0646-5

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335-344

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scholarly article

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10.1007/S00401-010-0646-5

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3

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119

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Ekaterina Rogaeva

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1029455786

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20111867

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amyotrophic lateral sclerosis