Computational simulation analysis on human SOD1 mutant (H80R) exposes the structural destabilization and the deviation of Zn binding that directs familial amyotrophic lateral sclerosis

Molecular dynamics simulations along with the computational predictions used to assess the protein structural characterization as well as the conformational preferences of the monomeric native and mutant SOD1.

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Formation of advanced glycation end-product-modified superoxide dismutase-1 (SOD1) is one of the mechanisms responsible for inclusions common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutation, and transgenic mice expressing human SOD1 gene mutation

Neuropathology ◽

10.1046/j.1440-1789.2001.00359.x ◽

2001 ◽

Vol 21 (1) ◽

pp. 67-81 ◽

Cited By ~ 21

Author(s):

Shinsuke Kato ◽

Kenji Nakashima ◽

Seikoh Horiuchi ◽

Ryoji Nagai ◽

Don W. Cleveland ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase ◽

Gene Mutation ◽

Familial Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase 1 ◽

Advanced Glycation ◽

Sod1 Gene ◽

Advanced Glycation End Product ◽

Human Sod1 ◽

Lateral Sclerosis

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Structural consequences of the familial amyotrophic lateral sclerosis SOD1 mutant His46Arg

Protein Science ◽

10.1110/ps.041256705 ◽

2005 ◽

Vol 14 (5) ◽

pp. 1201-1213 ◽

Cited By ~ 54

Author(s):

Svetlana Antonyuk ◽

Jennifer Stine Elam ◽

Michael A. Hough ◽

Richard W. Strange ◽

Peter A. Doucette ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Familial Amyotrophic Lateral Sclerosis ◽

Sod1 Mutant ◽

Lateral Sclerosis

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Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis

Neuroscience Letters ◽

10.1016/j.neulet.2016.05.006 ◽

2016 ◽

Vol 624 ◽

pp. 47-52 ◽

Cited By ~ 4

Author(s):

Xavier Gallart-Palau ◽

Chee-Hoe Ng ◽

Joan Ribera ◽

Siu Kwan Sze ◽

Kah-Leong Lim

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Familial Amyotrophic Lateral Sclerosis ◽

Phenotypic Features ◽

Human Sod1 ◽

Lateral Sclerosis

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Formation of advanced glycation end‐product‐modified superoxide dismutase‐1 (SOD1) is one of the mechanisms responsible for inclusions common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutation, and transgenic mice expressing human SOD1 gene mutation

Neuropathology ◽

10.1111/j.1440-1789.2001.00359.x ◽

2001 ◽

Vol 21 (1) ◽

pp. 67-81 ◽

Cited By ~ 1

Author(s):

Shinsuke Kato ◽

Kenji Nakashima ◽

Seikoh Horiuchi ◽

Ryoji Nagai ◽

Don W. Cleveland ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase ◽

Gene Mutation ◽

Familial Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase 1 ◽

Advanced Glycation ◽

Sod1 Gene ◽

Advanced Glycation End Product ◽

Human Sod1 ◽

Lateral Sclerosis

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Advanced glycation endproduct-modified superoxide dismutase-1 (SOD1)-positive inclusions are common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutations and transgenic mice expressing human SOD1 with a G85R mutation

Acta Neuropathologica ◽

10.1007/s004010000226 ◽

2000 ◽

Vol 100 (5) ◽

pp. 490-505 ◽

Cited By ~ 48

Author(s):

S. Kato ◽

Seiko Horiuchi ◽

Jian Liu ◽

Don W. Cleveland ◽

Noriyuki Shibata ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase ◽

Gene Mutations ◽

Familial Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase 1 ◽

Advanced Glycation ◽

Sod1 Gene ◽

Advanced Glycation Endproduct ◽

Human Sod1 ◽

Lateral Sclerosis

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Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation

Neuropathology ◽

10.1046/j.1440-1789.2001.00361.x ◽

2001 ◽

Vol 21 (1) ◽

pp. 82-92 ◽

Cited By ~ 72

Author(s):

Noriyuki Shibata

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase ◽

Mouse Model ◽

Transgenic Mouse ◽

Transgenic Mouse Model ◽

Familial Amyotrophic Lateral Sclerosis ◽

Superoxide Dismutase 1 ◽

Lateral Sclerosis

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Faculty Opinions recommendation of Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1022215.14073074 ◽

2011 ◽

Author(s):

Guy Rouleau

Keyword(s):

Spinal Cord ◽

Amyotrophic Lateral Sclerosis ◽

Mutant Proteins ◽

Sod1 Mutant ◽

Lateral Sclerosis

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389. Engineered microRNAs Against a C9ORF72 Transgenic Mouse Model To Treat Human Familial Amyotrophic Lateral Sclerosis (FALS)

Molecular Therapy ◽

10.1016/s1525-0016(16)35402-8 ◽

2014 ◽

Vol 22 ◽

pp. S148

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Mouse Model ◽

Transgenic Mouse ◽

Transgenic Mouse Model ◽

Familial Amyotrophic Lateral Sclerosis ◽

Lateral Sclerosis

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The densitometric physical fractionator for counting neuronal populations: application to a mouse model of familial amyotrophic lateral sclerosis

Journal of Neuroscience Methods ◽

10.1016/s0165-0270(03)00201-2 ◽

2003 ◽

Vol 129 (1) ◽

pp. 61-71 ◽

Cited By ~ 9

Author(s):

Giuseppe Luca Ciavarro ◽

Novella Calvaresi ◽

Andrea Botturi ◽

Caterina Bendotti ◽

Giuseppe Andreoni ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Mouse Model ◽

Familial Amyotrophic Lateral Sclerosis ◽

Neuronal Populations ◽

Lateral Sclerosis

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