X
Search Filters
Format Format
Subjects Subjects
Subjects Subjects
X
Sort by Item Count (A-Z)
Filter by Count
animals (58) 58
index medicus (51) 51
mice (45) 45
humans (40) 40
sandhoff-disease (31) 31
disease models, animal (26) 26
sandhoff disease (24) 24
mouse models (23) 23
biochemistry & molecular biology (20) 20
male (19) 19
female (18) 18
medicine, research & experimental (18) 18
brain (17) 17
enzymes (17) 17
sandhoff disease - pathology (17) 17
sandhoff disease - genetics (16) 16
analysis (15) 15
gangliosides (15) 15
gangliosidosis (15) 15
genetics & heredity (15) 15
mice, knockout (15) 15
neurodegeneration (15) 15
tay-sachs (15) 15
bone-marrow-transplantation (14) 14
immunology (14) 14
medicine (14) 14
neurology (14) 14
sandhoff disease - metabolism (14) 14
apoptosis (13) 13
mice, inbred c57bl (13) 13
multidisciplinary sciences (13) 13
mutation (13) 13
neurosciences (13) 13
research article (13) 13
gm2 gangliosidosis (12) 12
science (12) 12
biochemistry (11) 11
central-nervous-system (11) 11
g ganglioside - metabolism (11) 11
mouse model (11) 11
proteins (11) 11
tay-sachs disease (11) 11
tay-sachs-disease (11) 11
beta-n-acetylhexosaminidases - metabolism (10) 10
biotechnology & applied microbiology (10) 10
disease (10) 10
gene therapy (10) 10
n-butyldeoxynojirimycin (10) 10
biology (9) 9
cells, cultured (9) 9
expression (9) 9
ganglioside gm2 (9) 9
gaucher-disease (9) 9
genes (9) 9
lysosomal storage diseases (9) 9
metabolism (9) 9
sandhoff disease - immunology (9) 9
accumulation (8) 8
beta-n-acetylhexosaminidases - genetics (8) 8
brain - pathology (8) 8
central nervous system (8) 8
genetics (8) 8
glycosphingolipids - metabolism (8) 8
human tay-sachs (8) 8
lipids (8) 8
lysosomal storage disease (8) 8
lysosomes - metabolism (8) 8
mice, transgenic (8) 8
pathology (8) 8
sandhoff disease - therapy (8) 8
1-deoxynojirimycin - analogs & derivatives (7) 7
allergy and immunology (7) 7
article (7) 7
beta-hexosaminidase beta chain - genetics (7) 7
beta-hexosaminidase beta chain - metabolism (7) 7
brain - metabolism (7) 7
cell biology (7) 7
cells (7) 7
dependovirus - genetics (7) 7
fibroblasts (7) 7
gene expression (7) 7
lysosomal storage (7) 7
lysosomes (7) 7
nervous system (7) 7
nervous system diseases (7) 7
neurons (7) 7
neurons - pathology (7) 7
pharmacology (7) 7
research (7) 7
substrate reduction therapy (7) 7
adult (6) 6
beta-hexosaminidase (6) 6
cats (6) 6
gangliosidoses, gm2 - pathology (6) 6
genetic aspects (6) 6
genetic therapy (6) 6
immunohistochemistry (6) 6
infant (6) 6
inflammation (6) 6
phenotype (6) 6
more...
Language Language
Publication Date Publication Date
Click on a bar to filter by decade
Slide to change publication date range


Neuroscience, ISSN 0306-4522, 2016, Volume 340, pp. 117 - 125
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
European Journal of Immunology, ISSN 0014-2980, 10/2009, Volume 39, Issue 10, pp. 2748 - 2754
A defect in invariant NKT (iNKT) cell selection was hypothesized in lysosomal storage disorders (LSD). Accumulation of glycosphingolipids (GSL) in LSD could... 
Invariant NKT cells | Lysosomal storage disorders | Selecting ligand | MULTIPLE SULFATASE DEFICIENCY | MOLECULAR-GENETICS | METACHROMATIC LEUKODYSTROPHY | ANTIGEN PRESENTATION | ISOGLOBOTRIHEXOSYLCERAMIDE | IMMUNOLOGY | METABOLISM | MOUSE MODEL | KILLER T-CELLS | MICE | KRABBE-DISEASE | Leukodystrophy, Metachromatic - immunology | Liver - pathology | Sandhoff Disease - pathology | Spleen - immunology | Cell Count | Male | Leukodystrophy, Globoid Cell - immunology | Liver - immunology | Lysosomal Storage Diseases - immunology | Multiple Sulfatase Deficiency Disease - pathology | Natural Killer T-Cells - pathology | Thymus Gland - pathology | Female | Spleen - pathology | Disease Models, Animal | Multiple Sulfatase Deficiency Disease - immunology | Mice, Inbred C57BL | Enzyme Inhibitors - pharmacology | Leukodystrophy, Globoid Cell - pathology | Lysosomal Storage Diseases - genetics | Sandhoff Disease - genetics | Mice, Inbred Strains | Receptor, Fibroblast Growth Factor, Type 1 - antagonists & inhibitors | Mice, Knockout | Multiple Sulfatase Deficiency Disease - genetics | Sandhoff Disease - immunology | Cell Differentiation - immunology | Leukodystrophy, Metachromatic - genetics | Lymphocytes - pathology | Mucopolysaccharidosis I - immunology | Pyrroles - pharmacology | Animals | Cell Differentiation - drug effects | Mucopolysaccharidosis I - genetics | Thymus Gland - immunology | Leukodystrophy, Globoid Cell - genetics | Mice | Natural Killer T-Cells - immunology
Journal Article
Journal Article
Journal Article
Journal Article
Journal of Clinical Investigation, ISSN 0021-9738, 01/2004, Volume 113, Issue 2, pp. 200 - 208
Mice containing a disruption of the Hexb gene have provided a useful model system for the study of the human lysosomal storage disorder known as Sandhoff... 
MEDICINE, RESEARCH & EXPERIMENTAL | GLUCOCEREBROSIDASE | FC-RECEPTORS | BONE-MARROW-TRANSPLANTATION | REPLACEMENT THERAPY | CENTRAL-NERVOUS-SYSTEM | MICE | LYSOSOMAL STORAGE DISEASES | HUMAN TAY-SACHS | SANDHOFF-DISEASE | MOUSE MODELS
Journal Article
BBA - Protein Structure, ISSN 0005-2795, 1979, Volume 580, Issue 1, pp. 1 - 9
The immunological characteristics of feline β-hexosaminidase (β-d-N-acetylglucosaminidase, EC 3.2.1.30) isozymes, Hex A and Hex B, were studied. Immunization... 
Lysosomal enzyme | Acetylglucosaminidase | GM 2 gangliosidosis | Enzyme replacement | Immunologic characteristics | gangliosidosis | GM | Rabbits | Cats | Liver - enzymology | Goats | Species Specificity | Humans | Immunodiffusion | Epitopes | Isoenzymes - immunology | Cross Reactions | Sandhoff Disease - immunology | Animals | Hexosaminidases - immunology | Immunoelectrophoresis
Journal Article
Journal Article
Journal Article
Journal Article