Keyword

ALS pathogenesis

Description: The biological processes that cause or contribute to amyotrophic lateral sclerosis, including motor-neuron degeneration and related cellular dysfunction.

Primary topic: Amyotrophic Lateral Sclerosis Research (94%)

OpenAlex ID: keywords/als-pathogenesis

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

1994 · Mark E. Gurney, Haifeng Pu, et al. · Science · 4,136

Decoding ALS: from genes to mechanism

2016 · J. Paul Taylor, Robert H. Brown, et al. · Nature · 2,071

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TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

2008 · Edor Kabashi, Paul N Valdmanis, et al. · Nature Genetics · 1,545

Amyotrophic lateral sclerosis: a clinical review

2020 · P. Masrori, P. Van Damme · European Journal of Neurology · 1,207

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Molecular biology of amyotrophic lateral sclerosis: insights from genetics

2006 · Piera Pasinelli, Robert H. Brown · Nature reviews. Neuroscience · 1,106

Pathological TDP‐43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations

2007 · Ian R. A. Mackenzie, Eileen H. Bigio, et al. · Annals of Neurology · 991

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The changing scene of amyotrophic lateral sclerosis

2013 · Wim Robberecht, Thomas Philips · Nature reviews. Neuroscience · 956

Evidence of Increased Oxidative Damage in Both Sporadic and Familial Amyotrophic Lateral Sclerosis

1997 · Robert J. Ferrante, Susan E. Browne, et al. · Journal of Neurochemistry · 808

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

2011 · Faisal Fecto · Archives of Neurology · 651

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

2009 · Siân C. Barber, Pamela J. Shaw · Free Radical Biology and Medicine · 617

The phenotypic variability of amyotrophic lateral sclerosis

2014 · Bart Swinnen, Wim Robberecht · Nature Reviews Neurology · 604

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Rethinking ALS: The FUS about TDP-43

2009 · Clotilde Lagier-Tourenne, Don W. Cleveland · Cell · 588

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Protein aggregation in amyotrophic lateral sclerosis

2013 · Anna M. Blokhuis, Ewout J. N. Groen, et al. · Acta Neuropathologica · 580

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Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosis

1993 · Francine Côté, Jean-François Collard, et al. · Cell · 552

Energy metabolism in amyotrophic lateral sclerosis

2010 · Luc Dupuis, Pierre-François Pradat, et al. · The Lancet Neurology · 538

Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria

2004 · Jian Liu, Concepción Lillo, et al. · Neuron · 506

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Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

2008 · B TURNER, K TALBOT · Progress in Neurobiology · 496

Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target

2006 · Siân C. Barber, Richard J. Mead, et al. · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · 476

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Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosis

1995 · Jean-François Collard, Francine Côté, et al. · Nature · 463

Amyotrophic Lateral Sclerosis

2001 · Jean-Pierre Julien · Cell · 435

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Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

2014 · Leslie I. Grad, Justin J. Yerbury, et al. · Proceedings of the National Academy of Sciences · 432

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Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

2005 · Makoto Urushitani, Attila Sik, et al. · Nature Neuroscience · 419

Immune dysregulation in amyotrophic lateral sclerosis: mechanisms and emerging therapies

2019 · David R Beers, Stanley H Appel · The Lancet Neurology · 389

Amyotrophic lateral sclerosis associated with mutations in the CuZn superoxide dismutase gene

2006 · Peter M. Andersen · Current Neurology and Neuroscience Reports · 368

A role for astrocytes in motor neuron loss in amyotrophic lateral sclerosis

2004 · Luis H. Barbeito, Mariana Pehar, et al. · Brain Research Reviews · 327

Works count: 1,136

Citation count: 56,810