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Carnitine is a pharmacological allosteric chaperone of the human lysosomal α-glucosidase.
Iacono R, Minopoli N, Ferrara MC, Tarallo A, Damiano C, Porto C, Strollo S, Roig-Zamboni V, Peluso G, Sulzenbacher G, Cobucci-Ponzano B, Parenti G, Moracci M. Iacono R, et al. Among authors: porto c. J Enzyme Inhib Med Chem. 2021 Dec;36(1):2068-2079. doi: 10.1080/14756366.2021.1975694. J Enzyme Inhib Med Chem. 2021. PMID: 34565280 Free PMC article.
Correction of oxidative stress enhances enzyme replacement therapy in Pompe disease.
Tarallo A, Damiano C, Strollo S, Minopoli N, Indrieri A, Polishchuk E, Zappa F, Nusco E, Fecarotta S, Porto C, Coletta M, Iacono R, Moracci M, Polishchuk R, Medina DL, Imbimbo P, Monti DM, De Matteis MA, Parenti G. Tarallo A, et al. Among authors: porto c. EMBO Mol Med. 2021 Nov 8;13(11):e14434. doi: 10.15252/emmm.202114434. Epub 2021 Oct 4. EMBO Mol Med. 2021. PMID: 34606154 Free PMC article.
A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.
Parenti G, Fecarotta S, la Marca G, Rossi B, Ascione S, Donati MA, Morandi LO, Ravaglia S, Pichiecchio A, Ombrone D, Sacchini M, Pasanisi MB, De Filippi P, Danesino C, Della Casa R, Romano A, Mollica C, Rosa M, Agovino T, Nusco E, Porto C, Andria G. Parenti G, et al. Among authors: porto c. Mol Ther. 2014 Nov;22(11):2004-12. doi: 10.1038/mt.2014.138. Epub 2014 Jul 23. Mol Ther. 2014. PMID: 25052852 Free PMC article.
Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease.
Porto C, Pisani A, Rosa M, Acampora E, Avolio V, Tuzzi MR, Visciano B, Gagliardo C, Materazzi S, la Marca G, Andria G, Parenti G. Porto C, et al. J Inherit Metab Dis. 2012 May;35(3):513-20. doi: 10.1007/s10545-011-9424-3. Epub 2011 Dec 21. J Inherit Metab Dis. 2012. PMID: 22187137
342 results