Search Results - "Cupo, Ryan R."

Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations by Cupo, Ryan R, Shorter, James

“…Cells have evolved specialized protein disaggregases to reverse toxic protein aggregation and restore protein functionality. In nonmetazoan eukaryotes, the…”
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Mitochondrial fusion dynamics is robust in the heart and depends on calcium oscillations and contractile activity by Eisner, Verónica, Cupo, Ryan R., Gao, Erhe, Csordás, György, Slovinsky, William S., Paillard, Melanie, Cheng, Lan, Ibetti, Jessica, Chen, S. R. Wayne, Chuprun, J. Kurt, Hoek, Jan B., Koch, Walter J., Hajnóczky, György

“…Mitochondrial fusion is thought to be important for supporting cardiac contractility, but is hardly detectable in cultured cardiomyocytes and is difficult to…”
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Heterozygous variants of CLPB are a cause of severe congenital neutropenia by Warren, Julia T., Cupo, Ryan R., Wattanasirakul, Peeradol, Spencer, David H., Locke, Adam E., Makaryan, Vahagn, Bolyard, Audrey Anna, Kelley, Merideth L., Kingston, Natalie L., Shorter, James, Bellanné-Chantelot, Christine, Donadieu, Jean, Dale, David C., Link, Daniel C.

“…Severe congenital neutropenia is an inborn disorder of granulopoiesis. Approximately one third of cases do not have a known genetic cause. Exome sequencing of…”
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Tuning Hsp104 specificity to selectively detoxify α-synuclein by Mack, Korrie L., Kim, Hanna, Barbieri, Edward M., Lin, JiaBei, Braganza, Sylvanne, Jackrel, Meredith E., DeNizio, Jamie E., Yan, Xiaohui, Chuang, Edward, Tariq, Amber, Cupo, Ryan R., Castellano, Laura M., Caldwell, Kim A., Caldwell, Guy A., Shorter, James

“…Hsp104 is an AAA+ protein disaggregase that solubilizes and reactivates proteins trapped in aggregated states. We have engineered potentiated Hsp104 variants…”
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Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3 by Cupo, Ryan R., Rizo, Alexandrea N., Braun, Gabriel A., Tse, Eric, Chuang, Edward, Gupta, Kushol, Southworth, Daniel R., Shorter, James

“…The AAA+ protein, Skd3 (human CLPB), solubilizes proteins in the mitochondrial intermembrane space, which is critical for human health. Skd3 variants with…”
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Increased mitochondrial nanotunneling activity, induced by calcium imbalance, affects intermitochondrial matrix exchanges by Lavorato, Manuela, Iyer, V. Ramesh, Dewight, Williams, Cupo, Ryan R., Debattisti, Valentina, Gomez, Ludovic, De la Fuente, Sergio, Zhao, Yan-Ting, Valdivia, Héctor H., Hajnóczky, György, Franzini-Armstrong, Clara

“…Exchanges of matrix contents are essential to the maintenance of mitochondria. Cardiac mitochondrial exchange matrix content in two ways: by direct contact…”
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Expression and Purification of Recombinant Skd3 (Human ClpB) Protein and Tobacco Etch Virus (TEV) Protease from Escherichia coli by Cupo, Ryan R, Shorter, James

“…Skd3 (encoded by human ) is a mitochondrial AAA+ protein comprised of an N-terminal ankyrin-repeat domain and a C-terminal HCLR-clade nucleotide-binding…”
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Decoding Skd3 (Human CLPB ): a Mitochondrial Protein Disaggregase Critical for Human Health by Cupo, Ryan R., Shorter, James

“…Abstract Protein folding is important for all life. Indeed, protein misfolding can result in catastrophic protein aggregation and toxicity. The pathways…”
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Structural and mechanistic studies of Skd3 (human CLPB) provide insight into disease-causing mutations and reveal unique AAA+ oligomerization by Rizo, Alexandrea, Cupo, Ryan R., Shorter, James, Southworth, Daniel R.

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Exploring and Enhancing Compartment-Specific Protein Disaggregases to Combat Neurodegenerative Diseases by Cupo, Ryan R., Augustine, Emily, Shorter, James

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Increased mitochondrial nanotunneling activity, induced by calcium imbalance, affects intermitochondrial matrix by Lavorato, Manuela, Iyer, V Ramesh, Dewight, Williams, Cupo, Ryan R, Debattisti, Valentina, Gomez, Ludovic, De la Fuente, Sergio, Zhao, Yan-Ting, Valdivia, Héctor H, Hajnóczky, György, Franzini-Armstrong, Clara

“…Exchanges of matrix contents are essential to the maintenance of mitochondria. Cardiac mitochondrial exchange matrix content in two ways: by direct contact…”
Get full text