X
Search Filters
Format Format
Subjects Subjects
Subjects Subjects
X
Sort by Item Count (A-Z)
Filter by Count
animals (85) 85
humans (76) 76
index medicus (75) 75
mice (72) 72
sandhoff-disease (53) 53
sandhoff disease (49) 49
disease models, animal (45) 45
neurosciences (41) 41
genetics & heredity (37) 37
mouse models (36) 36
biochemistry & molecular biology (35) 35
1-deoxynojirimycin - analogs & derivatives (34) 34
n-butyldeoxynojirimycin (33) 33
male (31) 31
tay-sachs (31) 31
enzymes (30) 30
gangliosides (30) 30
tay-sachs-disease (30) 30
medicine, research & experimental (26) 26
sandhoff disease - genetics (26) 26
substrate reduction therapy (26) 26
sandhoff disease - drug therapy (25) 25
tay-sachs disease (24) 24
bone-marrow-transplantation (23) 23
female (23) 23
lysosomal storage diseases (22) 22
mouse model (21) 21
1-deoxynojirimycin - therapeutic use (20) 20
analysis (20) 20
gaucher-disease (20) 20
lysosomal storage (20) 20
mice, knockout (20) 20
beta-n-acetylhexosaminidases - genetics (19) 19
central-nervous-system (19) 19
g ganglioside - metabolism (19) 19
gangliosidosis (18) 18
gm2 gangliosidosis (18) 18
brain (17) 17
brain - metabolism (17) 17
mice, inbred c57bl (17) 17
neurodegeneration (17) 17
neurology (17) 17
sandhoff disease - pathology (17) 17
disease (16) 16
gangliosides - metabolism (16) 16
lysosomal storage disorders (16) 16
nervous system diseases (16) 16
treatment outcome (16) 16
endocrinology & metabolism (15) 15
enzyme inhibitors - therapeutic use (15) 15
lysosomal storage disease (15) 15
pediatrics (15) 15
1-deoxynojirimycin - pharmacology (14) 14
biotechnology & applied microbiology (14) 14
clinical neurology (14) 14
enzyme replacement therapy (14) 14
glycosphingolipids - metabolism (14) 14
mutation (14) 14
sandhoff disease - metabolism (14) 14
sandhoff disease - therapy (14) 14
beta-n-acetylhexosaminidases - metabolism (13) 13
genetics (13) 13
lysosomes - metabolism (13) 13
research (13) 13
biochemistry, general (12) 12
care and treatment (12) 12
cell biology (12) 12
enzyme inhibitors - pharmacology (12) 12
glycosphingolipids (12) 12
rodents (12) 12
brain - drug effects (11) 11
expression (11) 11
gaucher disease (11) 11
inflammation (11) 11
lipids (11) 11
miglustat (11) 11
pharmacology (11) 11
sandhoff disease - enzymology (11) 11
adult (10) 10
animals, newborn (10) 10
article (10) 10
glucosyltransferases - antagonists & inhibitors (10) 10
molecular biology (10) 10
physiological aspects (10) 10
sandhoff-disease mice (10) 10
tay-sachs disease - drug therapy (10) 10
beta-hexosaminidase (9) 9
cells, cultured (9) 9
dose-response relationship, drug (9) 9
drug discovery (9) 9
g gangliosidosis (9) 9
glucosylceramide synthase (9) 9
glycolipid biosynthesis (9) 9
gm1 gangliosidosis (9) 9
health aspects (9) 9
lysosomal storage disorder (9) 9
medicine & public health (9) 9
metabolism (9) 9
pathogenesis (9) 9
pathology (9) 9
more...
Library Location Library Location
Language Language
Publication Date Publication Date
Click on a bar to filter by decade
Slide to change publication date range


Journal Article
Annals of Neurology, ISSN 0364-5134, 04/2011, Volume 69, Issue 4, pp. 691 - 701
Journal Article
Brain, ISSN 0006-8950, 2012, Volume 135, Issue 6, pp. 1724 - 1735
Gaucher's disease, the most common lysosomal storage disorder, is caused by the defective activity of glucocerebrosidase, the lysosomal hydrolase that degrades... 
neuroinflammation | lysosomal storage disorders | Gaucher's disease | CYTOKINES | MICROGLIAL ACTIVATION | NEURODEGENERATION | SANDHOFF-DISEASE | NEUROSCIENCES | MOUSE MODELS | CLINICAL NEUROLOGY | PATHOPHYSIOLOGY | HEPARAN-SULFATE | MICE | TNF-ALPHA | Enzyme-Linked Immunosorbent Assay - methods | Ibuprofen - therapeutic use | Neurons - pathology | Encephalitis - diagnosis | Nestin | Blood-Brain Barrier - physiopathology | Gene Expression Regulation, Developmental - genetics | Tyrosine - analogs & derivatives | Antigens, CD - metabolism | Dose-Response Relationship, Drug | Glucosylceramidase - deficiency | Encephalitis - drug therapy | Intermediate Filament Proteins - genetics | Microglia - pathology | Microfilament Proteins - metabolism | Cell Death - drug effects | Neurons - drug effects | Cytokines - genetics | Gaucher Disease - drug therapy | Disease Models, Animal | Animals, Newborn | Calcium-Binding Proteins - metabolism | Cytokines - metabolism | Encephalitis - pathology | Mice, Transgenic | Gaucher Disease - complications | Nerve Tissue Proteins - genetics | Magnetic Resonance Imaging | Intercellular Adhesion Molecule-1 - metabolism | Tyrosine - metabolism | Animals | Gaucher Disease - genetics | Anti-Inflammatory Agents, Non-Steroidal - therapeutic use | Antigens, Differentiation, Myelomonocytic - metabolism | TATA Box | Mice | Endothelial Cells - pathology | Vascular Cell Adhesion Molecule-1 - metabolism | Encephalitis - etiology | Immunoglobulin G - metabolism
Journal Article
Brain, ISSN 0006-8950, 04/2003, Volume 126, Issue 4, pp. 974 - 987
Journal Article
Nature Medicine, ISSN 1078-8956, 04/2007, Volume 13, Issue 4, pp. 439 - 447
Journal Article
PLoS ONE, ISSN 1932-6203, 2011, Volume 6, Issue 6, p. e21758
Journal Article