Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis
about
Characterization of a human core-specific lysosomal {alpha}1,6-mannosidase involved in N-glycan catabolism.Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidased-PET-controlled "off-on" Polarity-sensitive Probes for Reporting Local Hydrophilicity within LysosomesCerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.Exploration and visualization of gene expression with neuroanatomy in the adult mouse brainImpaired lysosomal trimming of N-linked oligosaccharides leads to hyperglycosylation of native lysosomal proteins in mice with alpha-mannosidosis.Distribution and Severity of Neuropathology in β-Mannosidase-Deficient Mice is Strain Dependent.Latent herpes simplex virus infection of sensory neurons alters neuronal gene expression.A review of gene therapy in canine and feline models of lysosomal storage disorders.Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis miceAccumulation of free oligosaccharides and tissue damage in cytosolic α-mannosidase (Man2c1)-deficient mice.Animal models for metabolic, neuromuscular and ophthalmological rare diseases.Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment.Identification of 83 novel alpha-mannosidosis-associated sequence variants: functional analysis of MAN2B1 missense mutations.Human α-mannosidase produced in transgenic tobacco plants is processed in human α-mannosidosis cell lines.Intracellular transport of human lysosomal alpha-mannosidase and alpha-mannosidosis-related mutants.A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease.Allogeneic hematopoietic SCT for alpha-mannosidosis: an analysis of 17 patients.Alpha-Mannosidosis: Therapeutic Strategies.
P2860
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P2860
Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis
description
1999 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 1999
@ast
im August 1999 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 1999/08/01)
@sk
vědecký článek publikovaný v roce 1999
@cs
wetenschappelijk artikel (gepubliceerd op 1999/08/01)
@nl
наукова стаття, опублікована в серпні 1999
@uk
научни чланак (објављен 1999/08/01)
@sr
name
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@ast
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@en
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@nl
type
label
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@ast
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@en
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@nl
prefLabel
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@ast
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@en
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@nl
P2093
P3181
P356
P1476
Targeted disruption of the lys ...... rm of human alpha-mannosidosis
@en
P2093
A. Orlacchio
D. Hartmann
K. von Figura
R. Lüllmann-Rauch
S. Stinchi
T. Beccari
P304
P3181
P356
10.1093/HMG/8.8.1365
P577
1999-08-01T00:00:00Z