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Journal Article
PLoS Pathogens, ISSN 1553-7366, 12/2012, Volume 8, Issue 12, p. e1003078
Journal Article
Annals of Neurology, ISSN 0364-5134, 04/2011, Volume 69, Issue 4, pp. 691 - 701
Journal Article
Journal of Comparative Pathology, ISSN 0021-9975, 02/2014, Volume 150, Issue 2-3, pp. 253 - 257
G gangliosidosis (Tay–Sachs disease) was diagnosed in 6- to 8-month-old pedigree Jacob lambs from two unrelated flocks presenting clinically with progressive... 
GM2 gangliosidosis | Jacob sheep | Tay–Sachs disease | Animals | Sheep Diseases - genetics | beta-Hexosaminidase alpha Chain - genetics | Sheep | Gangliosidoses, GM2 - pathology | Mutation | Gangliosidoses, GM2 - veterinary | Gangliosidoses, GM2 - genetics | Sheep Diseases - pathology
Journal Article
Veterinary Pathology, ISSN 0300-9858, 7/2011, Volume 48, Issue 4, pp. 807 - 813
Journal Article
Human Gene Therapy, ISSN 1043-0342, 06/2017, Volume 28, Issue 6, pp. 51 - 522
GM2 gangliosidoses, including Tay–Sachs disease and Sandhoff disease, are lysosomal storage disorders caused by deficiencies in β-N-acetylhexosaminidase (Hex).... 
Research Articles | hexosaminidase | gene therapy | adeno-associated virus | Tay-Sachs disease | intracranial delivery | AAV | LONG-TERM | MOUSE-BRAIN | MEDICINE, RESEARCH & EXPERIMENTAL | SINGLE INJECTION | SANDHOFF-DISEASE | VECTOR | DELIVERY | FELINE MODEL | BIOTECHNOLOGY & APPLIED MICROBIOLOGY | GENETICS & HEREDITY | NERVOUS-SYSTEM DISEASE | GENE-THERAPY | REDUCES LYSOSOMAL STORAGE | Dependovirus - genetics | Neurons - pathology | Thalamus - metabolism | White Matter - metabolism | Injections, Intraventricular | Macaca fascicularis | Genetic Vectors - adverse effects | Male | Thalamus - pathology | Gangliosidoses, GM2 - pathology | Necrosis - pathology | Protein Subunits - metabolism | Apathy | Gray Matter - metabolism | Female | Neurons - metabolism | beta-N-Acetylhexosaminidases - genetics | Transgenes | Gangliosidoses, GM2 - genetics | Dyskinesias - genetics | Disease Models, Animal | Protein Subunits - genetics | Gangliosidoses, GM2 - therapy | Gene Expression | Dependovirus - metabolism | Genetic Vectors - chemistry | beta-N-Acetylhexosaminidases - metabolism | Dyskinesias - pathology | Genetic Vectors - metabolism | Necrosis - metabolism | Dyskinesias - etiology | Dyskinesias - metabolism | White Matter - pathology | Protein Subunits - adverse effects | Animals | Necrosis - etiology | Gray Matter - pathology | Necrosis - genetics | beta-N-Acetylhexosaminidases - adverse effects | Gangliosidoses, GM2 - metabolism | Genetic Therapy - methods | Neuroimaging | Brain | Hex protein | Central nervous system | Lysosomal storage diseases | Ventricles (cerebral) | Monkeys | Substantia grisea | Necrosis | Proteins | Infusion | Neurotoxicity | Histopathology | Coding | Ataxia | Thalamus | Safety | Lesions | Leukocytes (eosinophilic) | Abnormalities | Injection | Magnetic resonance imaging | Sheep | Ventricle
Journal Article
PLoS ONE, ISSN 1932-6203, 2010, Volume 5, Issue 8, p. e12105
Background: Sandhoff disease is a lysosomal storage disorder characterized by the absence of beta-hexosaminidase and storage of GM2 ganglioside and related... 
DISORDER BATTEN-DISEASE | GLUTAMIC-ACID DECARBOXYLASE | MACROPHAGE-INFLAMMATORY PROTEIN-1-ALPHA | BONE-MARROW-TRANSPLANTATION | MULTIDISCIPLINARY SCIENCES | INNATE IMMUNE-RESPONSE | MURINE LYMPHOCYTE SUBPOPULATIONS | LYSOSOMAL STORAGE DISEASES | HUMAN TAY-SACHS | SANDHOFF-DISEASE | MOUSE MODELS | Sandhoff Disease - pathology | Humans | Cell Death - immunology | Receptors, IgG - deficiency | Sandhoff Disease - metabolism | Infant | Male | Gene Expression Profiling | Gangliosidoses, GM2 - pathology | Autoantibodies - biosynthesis | RNA, Messenger - metabolism | Receptors, IgG - metabolism | Autoimmunity - immunology | Thymus Gland - pathology | Gangliosidoses, GM2 - immunology | Thymus Gland - metabolism | Gangliosidoses, GM2 - genetics | Macrophages - immunology | Disease Models, Animal | beta-Hexosaminidase beta Chain - metabolism | Chemokine CXCL13 - genetics | Gene Expression Regulation - immunology | Macrophage Activation - immunology | RNA, Messenger - genetics | Sandhoff Disease - genetics | Disease Progression | Aging - pathology | Sandhoff Disease - immunology | Macrophages - metabolism | Animals | Thymus Gland - immunology | Mice | Atrophy - metabolism | Gangliosidoses, GM2 - metabolism | Autoimmunity | Membrane lipids | Nervous system diseases | DNA microarrays | Autoantibodies | Analysis | B cells | Plant lipids | T cells | Gene expression | Gangliosides | Disease | Aluminum | Pathogenesis | CD8 antigen | Genes | Immunoglobulin G | CXCL13 protein | Ganglioside GM2 | Nervous system | Lymphocytes T | Macrophages | Thymus | Alterations | Lymphocytes | Bone marrow | Deoxyribonucleic acid--DNA | Lupus | Immune response | Cytokines | Chemotaxis | CD4 antigen | Medicine | Pathology | Glycolipids | Immunogenicity | Mutation | Autoimmune diseases | Chemokines | Apoptosis | Deoxyribonucleic acid | DNA
Journal Article
Journal of Veterinary Internal Medicine, ISSN 0891-6640, 09/2010, Volume 24, Issue 5, pp. 1013 - 1019
Journal Article