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Journal Article
Neurochemistry International, ISSN 0197-0186, 2008, Volume 52, Issue 6, pp. 1125 - 1133
Sandhoff disease involves the CNS accumulation of ganglioside GM2 and asialo-GM2 (GA2) due to inherited defects in the β-subunit gene of β-hexosaminidase A and... 
Neurodegenerative disease | Gangliosidosis | Substrate reduction therapy (SRT) | Glycosphingolipids | β-Hexosaminidase | GM2 | neurodegenerative disease | gangliosidosis | BONE-MARROW-TRANSPLANTATION | BIOCHEMISTRY & MOLECULAR BIOLOGY | THIN-LAYER CHROMATOGRAPHY | LYSOSOMAL STORAGE DISEASES | SUBSTRATE REDUCTION THERAPY | HUMAN TAY-SACHS | NEUROSCIENCES | MOUSE MODELS | GLYCOSPHINGOLIPID BIOSYNTHESIS | DEPRIVATION THERAPY | beta-hexosaminidase | substrate reduction therapy (SRT) | ACID BETA-GALACTOSIDASE | glycosphingolipids | I GAUCHER-DISEASE | Brain Chemistry - drug effects | Nerve Degeneration - physiopathology | Sandhoff Disease - metabolism | G(M2) Ganglioside - metabolism | Brain - metabolism | 1-Deoxynojirimycin - therapeutic use | Neuraminidase - drug effects | beta-Hexosaminidase alpha Chain - genetics | Nerve Degeneration - prevention & control | Sandhoff Disease - drug therapy | Gangliosides - metabolism | 1-Deoxynojirimycin - analogs & derivatives | Disease Models, Animal | Animals, Newborn | Brain - physiopathology | Neuraminidase - metabolism | Treatment Outcome | Down-Regulation - drug effects | Brain Chemistry - physiology | Mice, Knockout | Brain - drug effects | Down-Regulation - physiology | Sandhoff Disease - physiopathology | 1-Deoxynojirimycin - pharmacology | Animals | G(M2) Ganglioside - genetics | Glycosphingolipids - metabolism | Mice | Nerve Degeneration - drug therapy | Infants (Newborn) | Physiological aspects | Gangliosides | Index Medicus
Journal Article
Journal Article
NEUROLOGY, ISSN 0028-3878, 08/1996, Volume 47, Issue 2, pp. 547 - 552
Late-onset G(M2) gangliosidosis is a variant form of Tay-Sachs disease characterized by onset of symptoms and signs in adolescence or in early adult life. The... 
EXTENSIVE HOMOLOGY | JEWS | FORM | DNA | BIOSYNTHESIS | HUMAN BETA-HEXOSAMINIDASE | ADULT GM2 GANGLIOSIDOSIS | IDENTIFICATION | TAY-SACHS-DISEASE | CLINICAL NEUROLOGY | SUBUNIT
Journal Article
Bioorganic & Medicinal Chemistry Letters, ISSN 0960-894X, 03/2019, Volume 29, Issue 6, pp. 832 - 835
Bisorbicillinol is an inhibitor of β-hexosaminidase release and TNF-α secretion from RBL-2H3 cells, the inhibitory mechanism involved inhibition of Lyn, a... 
Lyn | TNF-α | Bisorbicillinol | β-Hexosaminidase | RBL-2H3 cells | METABOLITES | CHEMISTRY, MEDICINAL | FUNGUS | TNF-alpha | KEY INTERMEDIATE | MAST-CELLS | CHEMISTRY, ORGANIC | VERTICILLIUM-INTERTEXTUM | BIOSYNTHESIS | beta-Hexosaminidase | SORBICILLINOL | TRICHODERMA-LONGIBRACHIATUM | Tyrosine | Phenols | Safety and security measures | Leukemia | Food
Journal Article
Journal Article
Journal Article
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