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Research Interests:
A major research project in my laboratory is aimed at understanding how oxidative stress, superoxide dismutase, and zinc are involved in Lou Gehrig’s disease, also known as amyotrophic lateral sclerosis (ALS).
ALS is a dreadful disease caused by the unexplained death of motor neurons that control the movement of all voluntary muscles. We have only about 500,000 motor neurons at birth that cannot be replaced. One known cause of ALS is mutations in the antioxidant enzyme copper/zinc superoxide dismutase that results in the loss of zinc binding and then copper binding from the enzyme, making it toxic to motor neurons.
One of our major projects in the laboratory focuses on delivering copper to the enzyme to refold it properly. To this end, we are using a copper compound called Cu-ATSM, a form of copper that accumulates in hypoxic tissue, to deliver copper. Not only does Cu-ATSM appear to work in laboratory animals, it has recently facilitated linical trials on its therapies on humans with ALS.
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论文共 181 篇作者统计合作学者相似作者
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James B. W. Hilton,Kai Kysenius, Jeffrey R. Liddell, Stephen W. Mercer,Bence Paul,Joseph S. Beckman,Catriona A. McLean,Anthony R. White,Paul S. Donnelly,Ashley I. Bush,Dominic J. Hare,Blaine R. Roberts,
Scientific reportsno. 1 (2024): 5929-5929
Molecular Neurodegenerationno. 1 (2024): 1-21
ACS Central Scienceno. 4 (2023): 816-835
ACS CENTRAL SCIENCEno. 4 (2023): 816-835
Cassandra N. Dennys,Florence Roussel,Rochelle Rodrigo,Xiaojin Zhang, Andrea Sierra Delgado,Annalisa Hartlaub, Asya Saelim‐Ector, Will Ray,Sarah Heintzman,Ashley Fox,Stephen J. Kolb,Joseph Beckman,
Science Advancesno. 18 (2022)
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