Zobrazeno 1 - 10
of 11
pro vyhledávání: '"G. Michael Preston"'
Autor:
Helena Costa-Verdera, Fanny Collaud, Christopher R. Riling, Pauline Sellier, Jayme M. L. Nordin, G. Michael Preston, Umut Cagin, Julien Fabregue, Simon Barral, Maryse Moya-Nilges, Jacomina Krijnse-Locker, Laetitia van Wittenberghe, Natalie Daniele, Bernard Gjata, Jeremie Cosette, Catalina Abad, Marcelo Simon-Sola, Severine Charles, Mathew Li, Marco Crosariol, Tom Antrilli, William J. Quinn, David A. Gross, Olivier Boyer, Xavier M. Anguela, Sean M. Armour, Pasqualina Colella, Giuseppe Ronzitti, Federico Mingozzi
Publikováno v:
Nature Communications, Vol 12, Iss 1, Pp 1-16 (2021)
Pompe disease is currently treated with enzyme replacement therapy (ERT) with recombinant human acid alpha-glucosidase (GAA). Here, the authors show hepatic-directed gene therapy with AAV vectors enhances GAA bioavailability compared with ERT, result
Externí odkaz:
https://doaj.org/article/ecdd7a66d5fe4b0e825ff6e616f835cf
Autor:
Catalina Abad, N. Danièle, Jayme M.L. Nordin, Giuseppe Ronzitti, Bernard Gjata, Helena Costa-Verdera, Simon Barral, Sean M. Armour, Marcelo Simon-Sola, Fanny Collaud, Marco Crosariol, Julien Fabregue, David A. Gross, Mathew Li, Jérémie Cosette, Laetitia van Wittenberghe, Pasqualina Colella, Maryse Moya-Nilges, G. Michael Preston, Christopher Riling, Xavier M. Anguela, Federico Mingozzi, Severine Charles, Pauline Sellier, Tom Antrilli, William J. Quinn, Umut Cagin, Jacomina Krijnse-Locker, Olivier Boyer
Publikováno v:
Nature Communications
Nature Communications, 2021, 12 (1), pp.6393. ⟨10.1038/s41467-021-26744-4⟩
Nature Communications, Vol 12, Iss 1, Pp 1-16 (2021)
Nature Communications, Nature Publishing Group, 2021, 12 (1), pp.6393. ⟨10.1038/s41467-021-26744-4⟩
Nature Communications, 2021, 12 (1), pp.6393. ⟨10.1038/s41467-021-26744-4⟩
Nature Communications, Vol 12, Iss 1, Pp 1-16 (2021)
Nature Communications, Nature Publishing Group, 2021, 12 (1), pp.6393. ⟨10.1038/s41467-021-26744-4⟩
Pompe disease (PD) is a severe neuromuscular disorder caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). PD is currently treated with enzyme replacement therapy (ERT) with intravenous infusions of recombinant human GAA (rhGAA)
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9a1664cfde76136fe6521768528975a1
https://hal.sorbonne-universite.fr/hal-03449262/document
https://hal.sorbonne-universite.fr/hal-03449262/document
Autor:
Christopher J. Guerriero, Olivia E Hilal, Andrew A. Augustine, Jeffrey L. Brodsky, Zhihao Sun, Grace D Burns, Karl-Richard Reutter, G. Michael Preston
Publikováno v:
FEBS Lett
Maintenance of the proteome (proteostasis) is essential for cellular homeostasis and prevents cytotoxic stress responses that arise from protein misfolding. However, little is known about how different types of misfolded proteins impact homeostasis,
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9185f4823e83a34e61633c69315eaa42
https://pubmed.ncbi.nlm.nih.gov/34358326
https://pubmed.ncbi.nlm.nih.gov/34358326
Autor:
G. Michael Preston, Samuel K. Estabrooks, Jeffrey L. Brodsky, W. Seth Horne, Halina M. Werner
Publikováno v:
Chembiochem
Ubiquitin (Ub) plays critical roles in myriad protein degradation and signaling networks in the cell. We report herein Ub mimetics based on backbones that blend natural and artificial amino acid units. The variants were prepared by a modular route ba
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4ce3bd9bd828a11a996ec55270f3d7bb
https://doi.org/10.1002/cbic.201900225
https://doi.org/10.1002/cbic.201900225
Autor:
Karl-Richard Reutter, Timothy D. Mackie, Hillary C. Cleveland-Rubeor, Christopher J. Guerriero, Kunio Nakatsukasa, Michael Grabe, G. Michael Preston, Andrew A. Augustine, Kurt F. Weiberth, Neville P. Bethel, Jeffrey L. Brodsky, Keith M. Callenberg
Publikováno v:
Molecular Biology of the Cell
Cdc48/p97 provides the energy to retrotranslocate integral membrane ERAD substrates. A series of ERAD substrates with increasingly hydrophobic transmembrane helices is used to show that retrotranslocation efficiency inversely correlates with hydropho
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::693c0c2d870443d291316c99e2327620
http://europepmc.org/articles/PMC5509421
http://europepmc.org/articles/PMC5509421
Autor:
Jeffrey L. Brodsky, Hanoch Senderowitz, Netaly Khazanov, Teresa M. Buck, Christopher J. Guerriero, Edward A. Fisher, G. Michael Preston, Lynley M. Doonan
Publikováno v:
Protein Sci
Misfolded proteins in the endoplasmic reticulum (ER) are selected for ER-associated degradation (ERAD). More than 60 disease-associated proteins are substrates for the ERAD pathway due to the presence of missense or nonsense mutations. In yeast, the
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b75f47cb22f7f824d6df609046bfed86
https://doi.org/10.1002/pro.3636
https://doi.org/10.1002/pro.3636
Autor:
Jeffrey L. Brodsky, G. Michael Preston
Publikováno v:
The Biochemical journal. 474(4)
The endoplasmic reticulum (ER) serves as a warehouse for factors that augment and control the biogenesis of nascent proteins entering the secretory pathway. In turn, this compartment also harbors the machinery that responds to the presence of misfold
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e5182444686b481ac7e6e97ab5110c9e
https://pubmed.ncbi.nlm.nih.gov/28159894
https://pubmed.ncbi.nlm.nih.gov/28159894
Autor:
Emily K. Cook, Alexa S. Jordahl, Thomas R. Kleyman, Megan E. Yates, Jeffrey L. Brodsky, G. Michael Preston, Teresa M. Buck
Publikováno v:
The Biochemical journal. 474(3)
In the kidney, the epithelial sodium channel (ENaC) regulates blood pressure through control of sodium and volume homeostasis, and in the lung, ENaC regulates the volume of airway and alveolar fluids. ENaC is a heterotrimer of homologous α-, β- and
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cdacd55593c91c1ba60afd5f18276420
https://pubmed.ncbi.nlm.nih.gov/27903760
https://pubmed.ncbi.nlm.nih.gov/27903760
Autor:
G. Michael Preston, Jeffrey L. Brodsky, Susan Michaelis, Christopher J. Guerriero, Meredith B. Metzger
Publikováno v:
Molecular Cell. 70:242-253.e6
Summary Misfolded proteins in the endoplasmic reticulum (ER) are destroyed by ER-associated degradation (ERAD). Although the retrotranslocation of misfolded proteins from the ER has been reconstituted, how a polypeptide is initially selected for ERAD
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ce0bc1c99ff700ea1b6d62229d115b2e
https://doi.org/10.1016/j.molcel.2018.03.016
https://doi.org/10.1016/j.molcel.2018.03.016
Autor:
Simon C. Watkins, Souvik Chakraborty, Perunthottathu K. Umasankar, Gerard Apodaca, Linton M. Traub, G. Michael Preston, Puneet Khandelwal
Publikováno v:
The Journal of biological chemistry. 289(25)
The AP-2 clathrin adaptor complex oversees endocytic cargo selection in two parallel but independent manners. First, by physically engaging peptide-based endocytic sorting signals, a subset of clathrin-dependent transmembrane cargo is directly collec
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9812abc2d0a335bd312f98c7a91e7e49
https://pubmed.ncbi.nlm.nih.gov/24798335
https://pubmed.ncbi.nlm.nih.gov/24798335