Search Results - "Grad, Leslie I."

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms by Grad, Leslie I., Yerbury, Justin J., Turner, Bradley J., Guest, William C., Pokrishevsky, Edward, O'Neill, Megan A., Yanai, Anat, Silverman, Judith M., Zeineddine, Rafaa, Corcoran, Lisa, Kumita, Janet R., Luheshi, Leila M., Yousefi, Masoud, Coleman, Bradley M., Hill, Andrew F., Plotkin, Steven S., Mackenzie, Ian R., Cashman, Neil R.

“…Amyotrophic lateral sclerosis (ALS) is predominantly sporadic, but associated with heritable genetic mutations in 5–10% of cases, including those in Cu/Zn…”
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Competitive organelle-specific adaptors recruit Vps13 to membrane contact sites by Bean, Björn D M, Dziurdzik, Samantha K, Kolehmainen, Kathleen L, Fowler, Claire M S, Kwong, Waldan K, Grad, Leslie I, Davey, Michael, Schluter, Cayetana, Conibear, Elizabeth

“…The regulated expansion of membrane contact sites, which mediate the nonvesicular exchange of lipids between organelles, requires the recruitment of additional…”
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From molecule to molecule and cell to cell: Prion-like mechanisms in amyotrophic lateral sclerosis by Grad, Leslie I, Fernando, Sarah M, Cashman, Neil R

“…Abstract Prions, self-proliferating infectious agents consisting of misfolded protein, are most often associated with aggressive neurodegenerative diseases in…”
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TDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashion by Pokrishevsky, Edward, Grad, Leslie I., Cashman, Neil R.

“…Amyotrophic lateral sclerosis (ALS), which appears to spread through the neuroaxis in a spatiotemporally restricted manner, is linked to heritable mutations in…”
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Intermolecular transmission of Superoxide dismutase 1 misfolding in living cells by Grad, Leslie I., Guest, Will C., Yanai, Anat, Pokrishevsky, Edward, O'Neill, Megan A., Gibbs, Ebrima, Semenchenko, Valentyna, Yousefi, Masoud, Wishart, David S., Plotkin, Steven S., Cashman, Neil R.

“…Human wild-type Superoxide dismutase-1 (wtSOD1) is known to coaggregate with mutant SOD1 in familial amyotrophic lateral sclerosis (FALS), in double transgenic…”
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Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis by Pokrishevsky, Edward, Grad, Leslie I, Yousefi, Masoud, Wang, Jing, Mackenzie, Ian R, Cashman, Neil R

“…Amyotrophic lateral sclerosis (ALS) is incurable and characterized by progressive paralysis of the muscles of the limbs, speech and swallowing, and respiration…”
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Disease Mechanisms in ALS: Misfolded SOD1 Transferred Through Exosome-Dependent and Exosome-Independent Pathways by Silverman, Judith M., Fernando, Sarah M., Grad, Leslie I., Hill, Andrew F., Turner, Bradley J., Yerbury, Justin J., Cashman, Neil R.

“…Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset neuromuscular degenerative disorder with a poorly defined etiology. ALS patients experience motor…”
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Clinical Spectrum of Amyotrophic Lateral Sclerosis (ALS) by Grad, Leslie I, Rouleau, Guy A, Ravits, John, Cashman, Neil R

“…Amyotrophic lateral sclerosis (ALS) is primarily characterized by progressive loss of motor neurons, although there is marked phenotypic heterogeneity between…”
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Introduction of an Additional Pathway for Lactate Oxidation in the Treatment of Lactic Acidosis and Mitochondrial Dysfunction in Caenorhabditis elegans by Leslie I. Grad, Leanne C. Sayles, Lemire, Bernard D., Schatz, Gottfried

“…Mitochondrial dysfunction, with an estimated incidence of 1 in 5,000 births, is associated with a wide variety of multisystem degenerative diseases. Among the…”
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Prion-like activity of Cu/Zn superoxide dismutase: Implications for amyotrophic lateral sclerosis by Grad, Leslie I, Cashman, Neil R

“…Neurodegenerative diseases belong to a larger group of protein misfolding disorders, known as proteinopathies. There is increasing experimental evidence…”
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Intercellular Prion-Like Conversion and Transmission of Cu/Zn Superoxide Dismutase (SOD1) in Cell Culture by Grad, Leslie I, Pokrishevsky, Edward, Cashman, Neil R

“…The prion hypothesis has extended to the fatal motor neuron disease, amyotrophic lateral sclerosis (ALS), as a means to explain the spatiotemporal spread of…”
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Exosome-dependent and independent mechanisms are involved in prion-like transmission of propagated Cu/Zn superoxide dismutase misfolding by Grad, Leslie I, Pokrishevsky, Edward, Silverman, Judith M, Cashman, Neil R

“…Amyotrophic lateral sclerosis (ALS), a fatal adult-onset degenerative neuromuscular disorder with a poorly defined etiology, progresses in an orderly…”
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CNS-derived extracellular vesicles from superoxide dismutase 1 (SOD1)G93A ALS mice originate from astrocytes and neurons and carry misfolded SOD1 by Silverman, Judith M., Christy, Darren, Shyu, Chih Cheih, Moon, Kyung-Mee, Fernando, Sarah, Gidden, Zoe, Cowan, Catherine M., Ban, Yuxin, Stacey, R. Greg, Grad, Leslie I., McAlary, Luke, Mackenzie, Ian R., Foster, Leonard J., Cashman, Neil R.

“…Extracellular vesicles (EVs) are secreted by myriad cells in culture and also by unicellular organisms, and their identification in mammalian fluids suggests…”
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Generalization of the Prion Hypothesis to Other Neurodegenerative Diseases: An Imperfect Fit by Guest, Will C., Silverman, J. Maxwell, Pokrishevsky, Edward, O'Neill, Megan A., Grad, Leslie I., Cashman, Neil R.

“…Protein misfolding diseases have been classically understood as diffuse errors in protein folding, with misfolded protein arising autonomously throughout a…”
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Mitochondrial complex I mutations in Caenorhabditis elegans produce cytochrome c oxidase deficiency, oxidative stress and vitamin-responsive lactic acidosis by Grad, Leslie I., Lemire, Bernard D.

“…Mitochondrial dysfunction, with an estimated incidence of 1 in 10 000 live births, is among the most common genetically determined conditions. Missense…”
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Riboflavin enhances the assembly of mitochondrial cytochrome c oxidase in C. elegans NADH-ubiquinone oxidoreductase mutants by Grad, Leslie I., Lemire, Bernard D.

“…Mitochondrial respiratory chain dysfunction is responsible for a large variety of early and late-onset diseases. NADH-ubiquinone oxidoreductase (complex I)…”
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Isolation and functional analysis of mitochondria from the nematode Caenorhabditis elegans by Grad, Leslie I, Sayles, Leanne C, Lemire, Bernard D

“…Mitochondria are essential organelles with central roles in diverse cellular processes such as apoptosis, energy production via oxidative phosphorylation, ion…”
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Misfolded superoxide dismutase 1 (SOD1) as a novel target for Alzheimer's disease by Grad, Leslie I, Donkin, James, O'Neill, Megan A, Wang, Jing, Scrocchi, Louise A, Wellington, Cheryl L, Cashman, Neil R

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Locally released retinoic acid repatterns the first branchial arch cartilages in vivo by Plant, Marnie R., MacDonald, Mary P., Grad, Leslie I., Ritchie, Steven J., Richman, Joy M.

“…The fates of cranial neural crest cells are unique compared to trunk neural crest. Cranial neural crest cells form bone and cartilage and ultimately these…”
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Mitochondrial Respiratory Chain Deficiency inCaenorhabditis elegans Results in Developmental Arrest and Increased Life Span by Tsang, William Y., Sayles, Leanne C., Grad, Leslie I., Pilgrim, David B., Lemire, Bernard D.

“…The growth and development of Caenorhabditis elegans are energy-dependent and rely on the mitochondrial respiratory chain (MRC) as the major source of ATP. The…”
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