alpha-Galactosidase A deficient mice: a model of Fabry disease
about
Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cellsLocal and global cerebral blood flow and glucose utilization in the alpha-galactosidase A knockout mouse model of Fabry diseaseLong-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cellsReduction of globotriaosylceramide in Fabry disease mice by substrate deprivationInfrared imaging microscopy of bone: illustrations from a mouse model of Fabry diseaseNeurological deficits and glycosphingolipid accumulation in saposin B deficient mice.Cardiomyopathy and response to enzyme replacement therapy in a male mouse model for Fabry diseaseSubstrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry diseaseBiomarkers for disease progression and AAV therapeutic efficacy in feline Sandhoff diseaseHigh resolution MRI anatomy of the cat brain at 3 Tesla.Induced pluripotent stem cells derived from mouse models of lysosomal storage disordersIonization and fragmentation of neutral and acidic glycosphingolipids with a Q-TOF mass spectrometer fitted with a MALDI ion source.[Globosides as key players in the pathophysiology of Shiga toxin-associated acute kidney failure and Fabry disease].Lysosomal alpha-galactosidase controls the generation of self lipid antigens for natural killer T cellsPossible role of transforming growth factor-β1 and vascular endothelial growth factor in Fabry disease nephropathyThe pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease.Lentivector Iterations and Pre-Clinical Scale-Up/Toxicity Testing: Targeting Mobilized CD34+ Cells for Correction of Fabry DiseaseCharacterization of Fabry mice treated with recombinant adeno-associated virus 2/8-mediated gene transfer.Affective and cognitive behavior in the alpha-galactosidase A deficient mouse model of Fabry diseaseMulti-system disorders of glycosphingolipid and ganglioside metabolismEditing of CD1d-bound lipid antigens by endosomal lipid transfer proteins.A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis.Long-term systemic therapy of Fabry disease in a knockout mouse by adeno-associated virus-mediated muscle-directed gene transfer.Depletion of globosides and isoglobosides fully reverts the morphologic phenotype of Fabry diseaseFabry disease - current treatment and new drug development.Pain related channels are differentially expressed in neuronal and non-neuronal cells of glabrous skin of fabry knockout male miceConvenient and reproducible in vivo gene transfer to mouse parotid glandsAdeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice.Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.Advances in the management of Anderson-Fabry disease: enzyme replacement therapy.The mousetrap: what we can learn when the mouse model does not mimic the human disease.Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients.α-Galactosidase A expressed in the salivary glands partially corrects organ biochemical deficits in the fabry mouse through endocrine traffickingPreselective gene therapy for Fabry disease.Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transferFabry disease: recent advances in enzyme replacement therapy.Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies.Carboxyl-terminal truncations alter the activity of the human α-galactosidase AComparative evaluation of alpha-galactosidase A infusions for treatment of Fabry disease.
P2860
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P2860
alpha-Galactosidase A deficient mice: a model of Fabry disease
description
1997 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
1997 թվականի մարտին հրատարակված գիտական հոդված
@hy
article publié dans les Procee ...... f the United States of America
@fr
artículu científicu espublizáu en 1997
@ast
im März 1997 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 1997/03/18)
@sk
vědecký článek publikovaný v roce 1997
@cs
wetenschappelijk artikel (gepubliceerd op 1997/03/18)
@nl
наукова стаття, опублікована в березні 1997
@uk
name
alpha-Galactosidase A deficient mice: a model of Fabry disease
@ast
alpha-Galactosidase A deficient mice: a model of Fabry disease
@en
alpha-Galactosidase A deficient mice: a model of Fabry disease
@nl
type
label
alpha-Galactosidase A deficient mice: a model of Fabry disease
@ast
alpha-Galactosidase A deficient mice: a model of Fabry disease
@en
alpha-Galactosidase A deficient mice: a model of Fabry disease
@nl
prefLabel
alpha-Galactosidase A deficient mice: a model of Fabry disease
@ast
alpha-Galactosidase A deficient mice: a model of Fabry disease
@en
alpha-Galactosidase A deficient mice: a model of Fabry disease
@nl
P2093
P2860
P3181
P356
P1476
alpha-Galactosidase A deficient mice: a model of Fabry disease
@en
P2093
A. B. Kulkarni
C. O. Cardarelli
G. J. Murray
G. Longenecker
J. M. Quirk
M. M. Gottesman
R. O. Brady
T. Ohshima
W. D. Swaim
P2860
P304
P3181
P356
10.1073/PNAS.94.6.2540
P407
P577
1997-03-18T00:00:00Z