Structural Basis of Pharmacological Chaperoning for Human β-Galactosidase

G(M1) gangliosidosis and Morquio B disease are autosomal recessive diseases caused by the defect in the lysosomal β-galactosidase (β-Gal), frequently related to misfolding and subsequent endoplasmic reticulum-associated degradation. Pharmacological chaperone (PC) therapy is a newly developed molecul...

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Détails bibliographiques
Auteurs principaux: Suzuki, Hironori, Ohto, Umeharu, Higaki, Katsumi, Mena-Barragán, Teresa, Aguilar-Moncayo, Matilde, Ortiz Mellet, Carmen, Nanba, Eiji, Garcia Fernandez, Jose M., Suzuki, Yoshiyuki, Shimizu, Toshiyuki
Format: Artigo
Langue:Inglês
Publié: American Society for Biochemistry and Molecular Biology 2014
Sujets:
Protein Structure and Folding
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4031513/
https://ncbi.nlm.nih.gov/pubmed/24737316
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M113.529529
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