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Article

Structural basis of pharmacological chaperoning for human β-galactosidase.

The Journal of biological chemistry - 23 May 2014

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

Abstract excerpt

GM1 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 molecular therapeutic approach by using small molecule ligands of the mutant enzyme that are able to promote the correct...

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Identifiers

PMID: 24737316
DOI: 10.1074/jbc.M113.529529
PMCID: PMC4031513

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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