The potential action of galactose as a "chemical chaperone": increase of beta galactosidase activity in fibroblasts from an adult GM1-gangliosidosis patient.
about
Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of studyEmerging novel concept of chaperone therapies for protein misfolding diseasesCandidate molecules for chemical chaperone therapy of GM1-gangliosidosis.Identification and characterization of pharmacological chaperones to correct enzyme deficiencies in lysosomal storage disorders.Ganglioside biochemistryGM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings.Glucocerebrosidase inhibitors for the treatment of Gaucher disease.Pharmacological chaperones for enzyme enhancement therapy in genetic diseases.Innovative strategies to treat protein misfolding in inborn errors of metabolism: pharmacological chaperones and proteostasis regulators.Glycomimetic-based pharmacological chaperones for lysosomal storage disorders: lessons from Gaucher, GM1-gangliosidosis and Fabry diseases.Fluorous iminoalditols act as effective pharmacological chaperones against gene products from GLB₁ alleles causing GM1-gangliosidosis and Morquio B disease.Defining the genetic basis of early onset hereditary spastic paraplegia using whole genome sequencingDetermination of Acid β-Galactosidase Activity: Methodology and Perspectives.
P2860
Q21202879-778494DA-F8EF-4378-B845-90BD2C23D8ACQ27002907-FF898733-B823-444B-8CE3-CAD28665F559Q34985010-28751D3A-413D-445F-BA42-9FF60E4749DBQ35007910-1FE0B696-9610-4785-82BB-256DBD88944DQ35361488-4D0C8E57-631F-45C9-BCEF-90B41E7E327FQ35534727-C713FBFA-A92D-4B52-B622-E70D294E578EQ38097869-1ADFD17C-D995-400F-A536-1631E8CCD7C7Q38162958-DED9BBA3-83C6-42BB-856D-26C8263FD482Q38200933-BE62E815-4A74-4726-9A78-3F8E3108C4D7Q38797648-21944AF0-5948-4246-B3D6-22CEEFEE4357Q39692896-C7BBEEAB-159C-4F8A-B1E3-D6D6B3D6CCE6Q41445152-6B90420F-4631-494F-AFEE-1670C7975F40Q41894704-4240661E-B9EA-4A3F-9047-185560E271F9
P2860
The potential action of galactose as a "chemical chaperone": increase of beta galactosidase activity in fibroblasts from an adult GM1-gangliosidosis patient.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh-hant
name
The potential action of galact ...... lt GM1-gangliosidosis patient.
@en
The potential action of galact ...... lt GM1-gangliosidosis patient.
@nl
type
label
The potential action of galact ...... lt GM1-gangliosidosis patient.
@en
The potential action of galact ...... lt GM1-gangliosidosis patient.
@nl
prefLabel
The potential action of galact ...... lt GM1-gangliosidosis patient.
@en
The potential action of galact ...... lt GM1-gangliosidosis patient.
@nl
P2093
P1476
The potential action of galact ...... lt GM1-gangliosidosis patient.
@en
P2093
Alessandra d'Azzo
Amelia Morrone
Anna Caciotti
Enrico Zammarchi
Maria Alice Donati
Renzo Guerrini
Rosa Salvioli
P304
P356
10.1016/J.EJPN.2008.03.004
P577
2008-06-20T00:00:00Z