AGUILAR MONCAYO , Matilde

TiO2 nanoparticles have been prepared by amine assisted sol–gel precipitation of Ti4+ aqueous solutions and further hydrothermal treatment. The effect of different starting acidic solution (nitric, chlorhydric and acetic acids) as well as the addition of triethylamine (TEA) at different pH has been widely investigated. It has been stated that...

Compuestos potenciadores de la actividad de glicosida sas mutantes, con actividad inhibidoras de enzimas gli cosidasas y también se refiere a un procedimiento para la activación de β-glucosidasa mutante (β-glucocerebrosi dasa) y β-galactosidasa mutante en pacientes que pade cen trastornos de almacenamiento lisosómico mediante la administración...

A molecular-diversity-oriented approach for the preparation of bicyclic sp2-iminosugar glycomimetics related to nojirimycin and galactonojirimycin is reported. The synthetic strategy takes advantage of the ability of endocyclic pseudoamide-type atoms in five-membered cyclic iso(thio)ureas and guanidines to undergo intramolecular nucleophilic...

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...

Lysosomal β-galactosidase (β-Gal) deficiency causes a group of disorders that include neuronopathic GM1 gangliosidosis and non-neuronopathic Morquio B disease. We have previously proposed the use of small molecule ligands of β-Gal as pharmacological chaperones (PCs) for the treatment of GM1 gangliosidosis brain pathology. Although it is still...

Cyclodextrin-based host-guest chemistry has been exploited to facilitate co-crystallization of recombinant human acid β-glucosidase (β-glucocerebrosidase, GlcCerase) with amphiphilic bicyclic nojirimycin analogues of the sp2-iminosugar type. Attempts to co-crystallize GlcCerase with 5-N,6-O-[N′-(n-octyl)iminomethylidene]nojirimycin (NOI-NJ) or...