Search Results - "Raffaella Magnoni"

Molecular chaperone disorders: defective Hsp60 in neurodegeneration by Bross, Peter, Magnoni, Raffaella, Bie, Anne Sigaard

“…Chaperonins, a subgroup of molecular chaperones, form ring-shaped structures and assist folding of proteins by enclosing them in their inner cavity. The…”
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Late onset motoneuron disorder caused by mitochondrial Hsp60 chaperone deficiency in mice by Magnoni, Raffaella, Palmfeldt, Johan, Christensen, Jane H, Sand, Majken, Maltecca, Francesca, Corydon, Thomas J, West, Mark, Casari, Giorgio, Bross, Peter

“…Abstract Cells rely on efficient protein quality control systems (PQCs) to maintain proper activity of mitochondrial proteins. As part of this system, the…”
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The heat shock protein amplifier arimoclomol improves refolding, maturation and lysosomal activity of glucocerebrosidase by Fog, Cathrine K., Zago, Paola, Malini, Erika, Solanko, Lukasz M., Peruzzo, Paolo, Bornaes, Claus, Magnoni, Raffaella, Mehmedbasic, Arnela, Petersen, Nikolaj H.T., Bembi, Bruno, Aerts, Johannes F.M.G., Dardis, Andrea, Kirkegaard, Thomas

“…Gaucher Disease is caused by mutations of the GBA gene which encodes the lysosomal enzyme acid beta-glucosidase (GCase). GBA mutations commonly affect GCase…”
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Genetic interaction between the m-AAA protease isoenzymes reveals novel roles in cerebellar degeneration by Martinelli, Paola, La Mattina, Veronica, Bernacchia, Andrea, Magnoni, Raffaella, Cerri, Federica, Cox, Gregory, Quattrini, Angelo, Casari, Giorgio, Rugarli, Elena I.

“…The mitochondrial m-AAA protease has a crucial role in axonal development and maintenance. Human mitochondria possess two m-AAA protease isoenzymes: a…”
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Haploinsufficiency of AFG3L2, the Gene Responsible for Spinocerebellar Ataxia Type 28, Causes Mitochondria-Mediated Purkinje Cell Dark Degeneration by Maltecca, Francesca, Magnoni, Raffaella, Cerri, Federica, Cox, Gregory A, Quattrini, Angelo, Casari, Giorgio

“…Paraplegin and AFG3L2 are ubiquitous nuclear-encoded mitochondrial proteins that form hetero-oligomeric paraplegin-AFG3L2 and homo-oligomeric AFG3L2 complexes…”
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Arimoclomol increases the transcription of lysosomal genes, including NPC1 and NPC2, to facilitate lysosomal function by Petersen, Nikolaj H.T., Klein, Pontus, Valacca, Cristina, Bie, Anne, Magnoni, Raffaella, Pedersen, Marianne T., Lampp, Andrea C., Fog, Cathrine K., Kirkegaard, Thomas T.

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Arimoclomol increases the transcription of lysosomal genes, including and , to facilitate lysosomal function by Petersen, Nikolaj H.T., Klein, Pontus, Valacca, Cristina, Bie, Anne, Magnoni, Raffaella, Pedersen, Marianne T., Lampp, Andrea C., Fog, Cathrine K., Kirkegaard, Thomas T.

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Arimoclomol, a clinical candidate for neuronopathic Gaucher disease, increases glucocerebrosidase activity through amplification of heat shock proteins by Kirkegaard, Thomas, Fog-Tonnesen, Cathrine Kolster, Zago, Paola, Malini, Erika, Solanko, Lukasz, Bornaes, Claus, Magnoni, Raffaella, Petersen, Nikolaj H.T., Bembi, Bruno, Dardis, Andrea

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The heat shock protein amplifier arimoclomol improves refolding, maturation and lysosomal activity of glucocerebrosidaseResearch in context by Cathrine K. Fog, Paola Zago, Erika Malini, Lukasz M. Solanko, Paolo Peruzzo, Claus Bornaes, Raffaella Magnoni, Arnela Mehmedbasic, Nikolaj H.T. Petersen, Bruno Bembi, Johannes F.M.G. Aerts, Andrea Dardis, Thomas Kirkegaard

“…Background: Gaucher Disease is caused by mutations of the GBA gene which encodes the lysosomal enzyme acid beta-glucosidase (GCase). GBA mutations commonly…”
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