GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
about
Animal models of GM2 gangliosidosis: utility and limitationsLarge animal models of rare genetic disorders: sheep as phenotypically relevant models of human genetic diseasePathology of GM2 gangliosidosis in Jacob sheepDramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseasesTargeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs diseaseBiomarkers for disease progression and AAV therapeutic efficacy in feline Sandhoff diseaseHigh resolution MRI anatomy of the cat brain at 3 Tesla.Effective gene therapy in an authentic model of Tay-Sachs-related diseasesGene transfer corrects acute GM2 gangliosidosis--potential therapeutic contribution of perivascular enzyme flowTherapeutic response in feline sandhoff disease despite immunity to intracranial gene therapy.Characterization of a new model of GM2-gangliosidosis (Sandhoff's disease) in Korat catsBis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses.Clinical utility of neuronal cells directly converted from fibroblasts of patients for neuropsychiatric disorders: studies of lysosomal storage diseases and channelopathyCharacterization of the molecular defect in a feline model for type II GM2-gangliosidosis (Sandhoff disease).Needs for animal models of human diseases of the nervous system.The pathology of the feline model of mucopolysaccharidosis IA review of gene therapy in canine and feline models of lysosomal storage disorders.Tomoregulin-1 prevents cardiac hypertrophy after pressure overload in mice by inhibiting TAK1-JNK pathways.In situ detection of GM1 and GM2 gangliosides using immunohistochemical and immunofluorescent techniques for auxiliary diagnosis of canine and feline gangliosidoses.The pathology of feline GM2 gangliosidosis.AAV-mediated gene delivery in a feline model of Sandhoff disease corrects lysosomal storage in the central nervous system.Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff diseaseEctopic dendrites occur only on cortical pyramidal cells containing elevated GM2 ganglioside in alpha-mannosidosis.A canine model of human alpha-L-iduronidase deficiency.Real-time PCR genotyping assay for GM2 gangliosidosis variant 0 in toy poodles and the mutant allele frequency in Japan.AAV-mediated gene delivery attenuates neuroinflammation in feline Sandhoff disease.Clinical and biochemical abnormalities in porcine GM2-gangliosidosis.Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome.Direct Intracranial Injection of AAVrh8 Encoding Monkey β-N-Acetylhexosaminidase Causes Neurotoxicity in the Primate Brain.Lysosomal storage diseases of animals: an essay in comparative pathology.Widespread correction of central nervous system disease after intracranial gene therapy in a feline model of Sandhoff disease.Exocytosis of residual bodies in a lysosomal storage disease.Laboratory diagnosis of canine GM2-gangliosidosis using blood and cerebrospinal fluid.Clinical and molecular analysis of GM2 gangliosidosis in two apparent littermate kittens of the Japanese domestic cat.Mucolipidosis type II in a domestic shorthair cat.
P2860
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P2860
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
description
1977 nî lūn-bûn
@nan
1977年の論文
@ja
1977年学术文章
@wuu
1977年学术文章
@zh-cn
1977年学术文章
@zh-hans
1977年学术文章
@zh-my
1977年学术文章
@zh-sg
1977年學術文章
@yue
1977年學術文章
@zh
1977年學術文章
@zh-hant
name
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@ast
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@en
type
label
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@ast
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@en
prefLabel
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@ast
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@en
P2093
P356
P1433
P1476
GM2 ganglioside lysosomal storage disease in cats with beta-hexosaminidase deficiency.
@en
P2093
P304
P356
10.1126/SCIENCE.404709
P407
P577
1977-05-01T00:00:00Z