Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis
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Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisGrowth differentiation factor 6 as a putative risk factor in neuromuscular degenerationCalcium dysregulation links ALS defective proteins and motor neuron selective vulnerabilityClinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosisIntrinsic disorder in proteins involved in amyotrophic lateral sclerosis.A novel SOD1-ALS mutation separates central and peripheral effects of mutant SOD1 toxicityProteasome activation is a mechanism for pyrazolone small molecules displaying therapeutic potential in amyotrophic lateral sclerosisGuanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis.Identification of a misfolded region in superoxide dismutase 1 that is exposed in amyotrophic lateral sclerosis.Characterization of FUS mutations in amyotrophic lateral sclerosis using RNA-Seq.Identification of human monoclonal antibodies specific for human SOD1 recognizing distinct epitopes and forms of SOD1.Altered intracellular localization of SOD1 in leukocytes from patients with sporadic amyotrophic lateral sclerosis.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.Early-stage treatment with Withaferin A reduces levels of misfolded superoxide dismutase 1 and extends lifespan in a mouse model of amyotrophic lateral sclerosis.Intrinsically disordered proteins and their (disordered) proteomes in neurodegenerative disordersEarly detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patientsMacrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.Modulation of SOD1 Subcellular Localization by Transfection with Wild- or Mutant-type SOD1 in Primary Neuron and Astrocyte Cultures from ALS Mice.Post-mortem brain pathology is related to declining respiratory function in community-dwelling older adults.TDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashionAn antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man studyIs SOD1 loss of function involved in amyotrophic lateral sclerosis?Single chain variable fragment antibodies block aggregation and toxicity induced by familial ALS-linked mutant forms of SOD1.Endogenous macrophage migration inhibitory factor reduces the accumulation and toxicity of misfolded SOD1 in a mouse model of ALS.Glutathionylation potentiates benign superoxide dismutase 1 variants to the toxic forms associated with amyotrophic lateral sclerosis.Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism.Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALSA nonsense mutation in mouse Tardbp affects TDP43 alternative splicing activity and causes limb-clasping and body tone defects.Additive amelioration of ALS by co-targeting independent pathogenic mechanisms.Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanismsBlood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice.An enhanced integrated stress response ameliorates mutant SOD1-induced ALS.Dendritic protein synthesis in the normal and diseased brain.Sirtuin deacetylases in neurodegenerative diseases of aging.Advances in cellular models to explore the pathophysiology of amyotrophic lateral sclerosis.Studying neurodegenerative diseases in culture models.An emerging role for misfolded wild-type SOD1 in sporadic ALS pathogenesis.Prion-like activity of Cu/Zn superoxide dismutase: implications for amyotrophic lateral sclerosis.Targeting TDP-43 in neurodegenerative diseases.A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis.
P2860
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P2860
Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis
description
2012 nî lūn-bûn
@nan
2012 թուականին հրատարակուած գիտական յօդուած
@hyw
2012 թվականին հրատարակված գիտական հոդված
@hy
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
name
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@ast
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@en
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@nl
type
label
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@ast
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@en
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@nl
prefLabel
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@ast
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@en
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@nl
P2093
P2860
P1433
P1476
Aberrant localization of FUS a ...... amyotrophic lateral sclerosis
@en
P2093
Edward Pokrishevsky
Ian R Mackenzie
Leslie I Grad
Masoud Yousefi
Neil R Cashman
P2860
P304
P356
10.1371/JOURNAL.PONE.0035050
P407
P577
2012-01-01T00:00:00Z