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Results: 1 to 20 of 205

1.

An intersubunit disulfide bond prevents in vitro aggregation of a superoxide dismutase-1 mutant linked to familial amytrophic lateral sclerosis.

Ray SS, Nowak RJ, Strokovich K, Brown RH Jr, Walz T, Lansbury PT Jr.

Biochemistry. 2004 May 4;43(17):4899-905.

PMID:
15109247
[PubMed - indexed for MEDLINE]
2.

Small-molecule-mediated stabilization of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants against unfolding and aggregation.

Ray SS, Nowak RJ, Brown RH Jr, Lansbury PT Jr.

Proc Natl Acad Sci U S A. 2005 Mar 8;102(10):3639-44. Epub 2005 Feb 28.

PMID:
15738401
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

FALS mutations in Cu, Zn superoxide dismutase destabilize the dimer and increase dimer dissociation propensity: a large-scale thermodynamic analysis.

Khare SD, Caplow M, Dokholyan NV.

Amyloid. 2006 Dec;13(4):226-35.

PMID:
17107883
[PubMed - indexed for MEDLINE]
4.

ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization.

DiDonato M, Craig L, Huff ME, Thayer MM, Cardoso RM, Kassmann CJ, Lo TP, Bruns CK, Powers ET, Kelly JW, Getzoff ED, Tainer JA.

J Mol Biol. 2003 Sep 19;332(3):601-15. Erratum in: J Mol Biol. 2003 Nov 14;334(1):175.

PMID:
12963370
[PubMed - indexed for MEDLINE]
5.

Common dynamical signatures of familial amyotrophic lateral sclerosis-associated structurally diverse Cu, Zn superoxide dismutase mutants.

Khare SD, Dokholyan NV.

Proc Natl Acad Sci U S A. 2006 Feb 28;103(9):3147-52. Epub 2006 Feb 17.

PMID:
16488975
[PubMed - indexed for MEDLINE]
Free PMC Article
6.
7.

Insights into Lou Gehrig's disease from the structure and instability of the A4V mutant of human Cu,Zn superoxide dismutase.

Cardoso RM, Thayer MM, DiDonato M, Lo TP, Bruns CK, Getzoff ED, Tainer JA.

J Mol Biol. 2002 Nov 22;324(2):247-56.

PMID:
12441104
[PubMed - indexed for MEDLINE]
8.

Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1.

Niwa J, Yamada S, Ishigaki S, Sone J, Takahashi M, Katsuno M, Tanaka F, Doyu M, Sobue G.

J Biol Chem. 2007 Sep 21;282(38):28087-95. Epub 2007 Jul 31.

PMID:
17666395
[PubMed - indexed for MEDLINE]
Free Article
9.

Inducible superoxide dismutase 1 aggregation in transgenic amyotrophic lateral sclerosis mouse fibroblasts.

Turner BJ, Lopes EC, Cheema SS.

J Cell Biochem. 2004 Apr 1;91(5):1074-84.

PMID:
15034941
[PubMed - indexed for MEDLINE]
10.

Structural changes to monomeric CuZn superoxide dismutase caused by the familial amyotrophic lateral sclerosis-associated mutation A4V.

Schmidlin T, Kennedy BK, Daggett V.

Biophys J. 2009 Sep 16;97(6):1709-18. doi: 10.1016/j.bpj.2009.06.043.

PMID:
19751676
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

Mutation-dependent polymorphism of Cu,Zn-superoxide dismutase aggregates in the familial form of amyotrophic lateral sclerosis.

Furukawa Y, Kaneko K, Yamanaka K, Nukina N.

J Biol Chem. 2010 Jul 16;285(29):22221-31. doi: 10.1074/jbc.M110.113597. Epub 2010 Apr 19.

PMID:
20404329
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

SOD1 and amyotrophic lateral sclerosis: mutations and oligomerization.

Banci L, Bertini I, Boca M, Girotto S, Martinelli M, Valentine JS, Vieru M.

PLoS One. 2008 Feb 27;3(2):e1677. doi: 10.1371/journal.pone.0001677.

PMID:
18301754
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation.

Furukawa Y, O'Halloran TV.

J Biol Chem. 2005 Apr 29;280(17):17266-74. Epub 2005 Feb 3.

PMID:
15691826
[PubMed - indexed for MEDLINE]
Free Article
14.

Copper chaperone for superoxide dismutase co-aggregates with superoxide dismutase 1 (SOD1) in neuronal Lewy body-like hyaline inclusions: an immunohistochemical study on familial amyotrophic lateral sclerosis with SOD1 gene mutation.

Kato S, Sumi-Akamaru H, Fujimura H, Sakoda S, Kato M, Hirano A, Takikawa M, Ohama E.

Acta Neuropathol. 2001 Sep;102(3):233-8.

PMID:
11585247
[PubMed - indexed for MEDLINE]
15.

Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS.

Karch CM, Prudencio M, Winkler DD, Hart PJ, Borchelt DR.

Proc Natl Acad Sci U S A. 2009 May 12;106(19):7774-9. doi: 10.1073/pnas.0902505106. Epub 2009 Apr 30.

PMID:
19416874
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Folding of Cu, Zn superoxide dismutase and familial amyotrophic lateral sclerosis.

Khare SD, Ding F, Dokholyan NV.

J Mol Biol. 2003 Nov 28;334(3):515-25.

PMID:
14623191
[PubMed - indexed for MEDLINE]
18.

Superoxide dismutase 1 subunits with mutations linked to familial amyotrophic lateral sclerosis do not affect wild-type subunit function.

Borchelt DR, Guarnieri M, Wong PC, Lee MK, Slunt HS, Xu ZS, Sisodia SS, Price DL, Cleveland DW.

J Biol Chem. 1995 Feb 17;270(7):3234-8.

PMID:
7852409
[PubMed - indexed for MEDLINE]
Free Article
19.

Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans.

Oeda T, Shimohama S, Kitagawa N, Kohno R, Imura T, Shibasaki H, Ishii N.

Hum Mol Genet. 2001 Sep 15;10(19):2013-23.

PMID:
11590119
[PubMed - indexed for MEDLINE]
Free Article
20.

Aberrantly increased hydrophobicity shared by mutants of Cu,Zn-superoxide dismutase in familial amyotrophic lateral sclerosis.

Tiwari A, Xu Z, Hayward LJ.

J Biol Chem. 2005 Aug 19;280(33):29771-9. Epub 2005 Jun 15.

PMID:
15958382
[PubMed - indexed for MEDLINE]
Free Article

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