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Journal Article
Journal Article
PLoS ONE, ISSN 1932-6203, 11/2011, Volume 6, Issue 12, pp. e29074 - e29074
Journal Article
Annals of Neurology, ISSN 0364-5134, 03/2011, Volume 69, Issue 4, pp. 691 - 701
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 | Index Medicus | Deoxyribonucleic acid | DNA
Journal Article
Journal Article
Molecular Therapy, ISSN 1525-0016, 03/2015, Volume 23, Issue 3, pp. 414 - 422
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 6/2004, Volume 101, Issue 22, pp. 8425 - 8430
Sandhoff disease is a prototypical lysosomal storage disorder in which a heritable deficiency of a lysosomal enzyme, β-hexosaminidase, results in the storage... 
Biological Sciences | Spinal cord | Nervous system diseases | Messenger RNA | Neurons | Astrocytes | Central nervous system | Central nervous system diseases | Lysosomal storage diseases | Mice | Sandhoff disease | CELLS | GANGLIOSIDOSIS | MICROGLIA | BONE-MARROW-TRANSPLANTATION | CHEMOKINES | MULTIDISCIPLINARY SCIENCES | CENTRAL-NERVOUS-SYSTEM | TAY-SACHS | MIP-1-ALPHA | BLOOD-BRAIN-BARRIER | MOUSE MODELS | Heredodegenerative Disorders, Nervous System - genetics | Up-Regulation | Body Weight | Central Nervous System - metabolism | Sandhoff Disease - pathology | Monocytes - cytology | Heredodegenerative Disorders, Nervous System - pathology | Humans | Sandhoff Disease - metabolism | Child, Preschool | Male | Monocytes - metabolism | Life Expectancy | Female | Macrophage-1 Antigen - metabolism | beta-N-Acetylhexosaminidases - genetics | Disease Models, Animal | Astrocytes - cytology | Chemokine CCL4 | Chemokine CCL3 | beta-N-Acetylhexosaminidases - metabolism | Macrophage Inflammatory Proteins - metabolism | Sandhoff Disease - genetics | Behavior, Animal - physiology | Macrophages - cytology | Macrophage Inflammatory Proteins - genetics | Mice, Knockout | Macrophages - metabolism | Animals | Central Nervous System - cytology | Macrophage-1 Antigen - genetics | Heredodegenerative Disorders, Nervous System - metabolism | Apoptosis | Astrocytes - metabolism | Index Medicus
Journal Article
Journal of Inborn Errors of Metabolism and Screening, ISSN 2214-6490, 3/2014, Volume 2, Issue 1-2, p. 1
Lysosomal storage diseases are a group of inherited and acquired disorders. They are characterized by interruption of recycling of cellular and extracellular... 
Journal Article