β-Glucose-1,6-Bisphosphate Stabilizes Pathological Phophomannomutase2 Mutants In Vitro and Represents a Lead Compound to Develop Pharmacological Chaperones for the Most Common Disorder of Glycosylation, PMM2-CDG

A large number of mutations causing PMM2-CDG, which is the most frequent disorder of glycosylation, destabilize phosphomannomutase2. We looked for a pharmacological chaperone to cure PMM2-CDG, starting from the structure of a natural ligand of phosphomannomutase2, α-glucose-1,6-bisphosphate. The com...

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Detalles Bibliográficos
Autores principales: Monticelli, Maria, Liguori, Ludovica, Allocca, Mariateresa, Andreotti, Giuseppina, Cubellis, Maria Vittoria
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6747070/
https://www.ncbi.nlm.nih.gov/pubmed/31454904
http://dx.doi.org/10.3390/ijms20174164

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6747070/
https://www.ncbi.nlm.nih.gov/pubmed/31454904
http://dx.doi.org/10.3390/ijms20174164

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