Search Results - "Doucette, Peter A"

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis by Valentine, Joan Selverstone, Doucette, Peter A, Zittin Potter, Soshanna

“…Copper-zinc superoxide dismutase (CuZnSOD, SOD1 protein) is an abundant copper- and zinc-containing protein that is present in the cytosol, nucleus,…”
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Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase by Oztug Durer, Zeynep A, Cohlberg, Jeffrey A, Dinh, Phong, Padua, Shelby, Ehrenclou, Krista, Downes, Sean, Tan, James K, Nakano, Yoko, Bowman, Christopher J, Hoskins, Jessica L, Kwon, Chuhee, Mason, Andrew Z, Rodriguez, Jorge A, Doucette, Peter A, Shaw, Bryan F, Valentine, Joan Selverstone

“…Mutations in the gene encoding Cu-Zn superoxide dismutase (SOD1) are one of the causes of familial amyotrophic lateral sclerosis (FALS). Fibrillar inclusions…”
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Variable Metallation of Human Superoxide Dismutase: Atomic Resolution Crystal Structures of Cu–Zn, Zn–Zn and As-isolated Wild-type Enzymes by Strange, Richard W., Antonyuk, Svetlana V., Hough, Michael A., Doucette, Peter A., Valentine, Joan Selverstone, Hasnain, S. Samar

“…Human Cu–Zn superoxide dismutase (SOD1) protects cells from the effects of oxidative stress. Mutations in SOD1 are linked to the familial form of amyotrophic…”
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Binding of a Single Zinc Ion to One Subunit of Copper−Zinc Superoxide Dismutase Apoprotein Substantially Influences the Structure and Stability of the Entire Homodimeric Protein by Potter, Soshanna Zittin, Zhu, Haining, Shaw, Bryan Francis, Rodriguez, Jorge A, Doucette, Peter A, Sohn, Se Hui, Durazo, Armando, Faull, Kym F, Gralla, Edith Butler, Nersissian, Aram M, Valentine, Joan Selverstone

“…The thermodynamics of zinc binding to metal-free (apo) human and bovine copper−zinc superoxide dismutases (SOD1) were measured using isothermal titration…”
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Destabilization of Apoprotein Is Insufficient to Explain Cu,Zn-Superoxide Dismutase-Linked ALS Pathogenesis by Rodriguez, Jorge A., Shaw, Bryan Francis, Durazo, Armando, Sohn, Se Hui, Doucette, Peter A., Nersissian, Aram M., Faull, Kym F., Eggers, Daryl K., Tiwari, Ashutosh, Hayward, Lawrence J., Valentine, Joan Selverstone, Fridovich, Irwin

“…The relative stabilities and structural properties of a representative set of 20 ALS-mutant Cu,Zn-superoxide dismutase apoproteins were examined by using…”
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Structures of the G85R Variant of SOD1 in Familial Amyotrophic Lateral Sclerosis by Cao, Xiaohang, Antonyuk, Svetlana V., Seetharaman, Sai V., Whitson, Lisa J., Taylor, Alexander B., Holloway, Stephen P., Strange, Richard W., Doucette, Peter A., Valentine, Joan Selverstone, Tiwari, Ashutosh, Hayward, Lawrence J., Padua, Shelby, Cohlberg, Jeffrey A., Hasnain, S. Samar, Hart, P. John

“…Mutations in the gene encoding human copper-zinc superoxide dismutase (SOD1) cause a dominant form of the progressive neurodegenerative disease amyotrophic…”
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The Structure of Holo and Metal-deficient Wild-type Human Cu, Zn Superoxide Dismutase and its Relevance to Familial Amyotrophic Lateral Sclerosis by Strange, Richard W., Antonyuk, Svetlana, Hough, Michael A., Doucette, Peter A., Rodriguez, Jorge A., Hart, P.John, Hayward, Lawrence J., Valentine, Joan S., Hasnain, S.Samar

“…Cu, Zn superoxide dismutase (SOD1) forms a crucial component of the cellular defence against oxidative stress. Zn-deficient wild-type and mutant human SOD1…”
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Dimer Destabilization in Superoxide Dismutase May Result in Disease-Causing Properties: Structures of Motor Neuron Disease Mutants by Hough, Michael A., Grossmann, J. Günter, Antonyuk, Svetlana V., Strange, Richard W., Doucette, Peter A., Rodriguez, Jorge A., Whitson, Lisa J., Hart, P. John, Hayward, Lawrence J., Valentine, Joan Selverstone, Hasnain, S. Samar, Petsko, Gregory A.

“…More than 90 point mutations in human CuZn superoxide dismutase lead to the development of familial amyotrophic lateral sclerosis, known also as motor neuron…”
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Structural consequences of the familial amyotrophic lateral sclerosis SOD1 mutant His46Arg by Antonyuk, Svetlana, Elam, Jennifer Stine, Hough, Michael A., Strange, Richard W., Doucette, Peter A., Rodriguez, Jorge A., Hayward, Lawrence J., Valentine, Joan Selverstone, Hart, P. John, Hasnain, S. Samar

“…The His46Arg (H46R) mutant of human copper‐zinc superoxide dismutase (SOD1) is associated with an unusual, slowly progressing form of familial amyotrophic…”
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Dissociation of human copper-zinc superoxide dismutase dimers using chaotrope and reductant. Insights into the molecular basis for dimer stability by Doucette, Peter A, Whitson, Lisa J, Cao, Xiaohang, Schirf, Virgil, Demeler, Borries, Valentine, Joan Selverstone, Hansen, Jeffrey C, Hart, P John

“…The dissociation of apo- and metal-bound human copper-zinc superoxide dismutase (SOD1) dimers induced by the chaotrope guanidine hydrochloride (GdnHCl) or the…”
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Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS by Hart, P John, Elam, Jennifer Stine, Taylor, Alexander B, Strange, Richard, Antonyuk, Svetlana, Doucette, Peter A, Rodriguez, Jorge A, Hasnain, S Samar, Hayward, Lawrence J, Valentine, Joan Selverstone, Yeates, Todd O

“…Mutations in the SOD1 gene cause the autosomal dominant, neurodegenerative disorder familial amyotrophic lateral sclerosis (FALS). In spinal cord neurons of…”
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Disrupted Zinc-Binding Sites in Structures of Pathogenic SOD1 Variants D124V and H80R by Seetharaman, Sai V, Winkler, Duane D, Taylor, Alexander B, Cao, Xiaohang, Whitson, Lisa J, Doucette, Peter A, Valentine, Joan S, Schirf, Virgil, Demeler, Borries, Carroll, Mark C, Culotta, Valeria C, Hart, P. John

“…Mutations in human copper−zinc superoxide dismutase (SOD1) cause an inherited form of the fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS)…”
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An alternative mechanism of bicarbonate-mediated peroxidation by copper-zinc superoxide dismutase: rates enhanced via proposed enzyme-associated peroxycarbonate intermediate by Elam, Jennifer Stine, Malek, Kevin, Rodriguez, Jorge A, Doucette, Peter A, Taylor, Alexander B, Hayward, Lawrence J, Cabelli, Diane E, Valentine, Joan Selverstone, Hart, P John

“…Hydrogen peroxide can interact with the active site of copper-zinc superoxide dismutase (SOD1) to generate a powerful oxidant. This oxidant can either damage…”
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Dissociation of Human Copper-Zinc Superoxide Dismutase Dimers Using Chaotrope and Reductant by Doucette, Peter A., Whitson, Lisa J., Cao, Xiaohang, Schirf, Virgil, Demeler, Borries, Valentine, Joan Selverstone, Hansen, Jeffrey C., Hart, P. John

“…The dissociation of apo- and metal-bound human copper-zinc superoxide dismutase (SOD1) dimers induced by the chaotrope guanidine hydrochloride (GdnHCl) or the…”
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An Alternative Mechanism of Bicarbonate-mediated Peroxidation by Copper-Zinc Superoxide Dismutase by Elam, Jennifer Stine, Malek, Kevin, Rodriguez, Jorge A., Doucette, Peter A., Taylor, Alexander B., Hayward, Lawrence J., Cabelli, Diane E., Valentine, Joan Selverstone, Hart, P. John

“…Hydrogen peroxide can interact with the active site of copper-zinc superoxide dismutase (SOD1) to generate a powerful oxidant. This oxidant can either damage…”
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Structures of the G85R Variant of SOD1 in Familial Amyotrophic Lateral SclerosisS by Cao, Xiaohang, Antonyuk, Svetlana V., Seetharaman, Sai V., Whitson, Lisa J., Taylor, Alexander B., Holloway, Stephen P., Strange, Richard W., Doucette, Peter A., Valentine, Joan Selverstone, Tiwari, Ashutosh, Hayward, Lawrence J., Padua, Shelby, Cohlberg, Jeffrey A., Hasnain, S. Samar, Hart, P. John

“…Mutations in the gene encoding human copper-zinc superoxide dismutase (SOD1) cause a dominant form of the progressive neurodegenerative disease amyotrophic…”
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Misfolded superoxide dismutase and ALS by Hart, P.John, Elam, Jennifer Stine, Taylor, Alexander B., Strange, Richard, Hasnain, S.Samar, Doucette, Peter A., Valentine, Joan S., Hayward, Lawrence J.

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