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Journal Article
PLoS ONE, ISSN 1932-6203, 04/2012, Volume 7, Issue 4, pp. e35787 - e35787
Mucopolysaccharide diseases (MPS) are caused by deficiency of glycosaminoglycan (GAG) degrading enzymes, leading to GAG accumulation. Neurodegenerative MPS... 
TARGETED DISRUPTION | MICROGLIAL ACTIVATION | SANFILIPPO-SYNDROME | DISEASE | BIOLOGY | SYNDROME TYPE-B | MICE | HEPARAN-SULFATE PROTEOGLYCANS | LYSOSOMAL STORAGE DISORDERS | ALPHA-L-IDURONIDASE | BRAIN | Immunohistochemistry | Neurons - pathology | Mucopolysaccharidosis III - pathology | Male | Parietal Lobe - metabolism | Heparitin Sulfate - metabolism | Somatosensory Cortex - pathology | Lysosomes - metabolism | Homer Scaffolding Proteins | Mucopolysaccharidosis III - metabolism | Female | Lysosomes - pathology | Neurons - metabolism | G(M2) Ganglioside - biosynthesis | Disease Models, Animal | Glycosaminoglycans - biosynthesis | Mucopolysaccharidosis I - metabolism | Mucopolysaccharidosis I - pathology | Carrier Proteins - biosynthesis | Disease Progression | Parietal Lobe - pathology | Animals | Vesicle-Associated Membrane Protein 2 - biosynthesis | Mice | Cytokines - biosynthesis | Somatosensory Cortex - metabolism | Enzymes | Brain | Neurons | Analysis | Models | Gangliosides | Animal models | Gangliosidosis | Transplants & implants | Disease | Neuropathology | Genes | Cognitive ability | Ganglioside GM2 | Schizophrenia | Cortex (somatosensory) | Mucopolysaccharidosis | Cortex (motor) | Proteins | Blood-brain barrier | Neurodegeneration | Cortex (parietal) | Rodents | Fibroblasts | Bone marrow | Growth factors | Genotypes | Heparan sulfate | Synaptophysin | Inflammation | Electron microscopy | Sulfation | Membrane proteins | Pathology | Axons | Gliosis | Hospitals | Transmission electron microscopy | Acids | Life span | Cell number | Stem cells | Monocyte chemoattractant protein 1 | Index Medicus
Journal Article
Journal of Neuroimmunology, ISSN 0165-5728, 2009, Volume 209, Issue 1, pp. 139 - 142
Journal Article
Journal Article
Journal Article
Journal Article
Journal of Neurochemistry, ISSN 0022-3042, 09/2005, Volume 94, Issue 6, pp. 1631 - 1638
Sandhoff disease is an autosomal recessive lysosomal storage disease caused by a defect of the β‐subunit gene ( HEXB ) associated with simultaneous... 
N‐acetylglucosaminyl oligosaccharides | enzyme replacement therapy | β‐hexosmanidase A | Sandhoff disease | GM2 ganglioside | lentiviral vector | N-acetylglucosaminyl oligosaccharides | Lentiviral vector | β-hexosmanidase A | Enzyme replacement therapy | ACTIVATION | PROTEIN | GANGLIOSIDOSIS | ALZHEIMERS-DISEASE | BIOCHEMISTRY & MOLECULAR BIOLOGY | NEUROSCIENCES | MOUSE MODELS | beta-hexosmanidase A | PATHOGENESIS | MACROPHAGE-INFLAMMATORY PROTEIN-1-ALPHA | GENE-EXPRESSION | CENTRAL-NERVOUS-SYSTEM | TAY-SACHS | Microglia - metabolism | Humans | Sandhoff Disease - metabolism | Brain - virology | Male | Gliosis - physiopathology | Genetic Vectors - therapeutic use | G(M2) Ganglioside - metabolism | Receptor, IGF Type 2 - metabolism | Brain - metabolism | Lentivirus - metabolism | Encephalitis - physiopathology | Encephalitis - metabolism | Sandhoff Disease - therapy | Female | Lentivirus - genetics | beta-N-Acetylhexosaminidases - genetics | Dimerization | Disease Models, Animal | Protein Subunits - genetics | Calcium-Binding Proteins - metabolism | Hexosaminidase B | Gliosis - genetics | Encephalitis - genetics | Hexosaminidase A | Isoenzymes - genetics | Brain - physiopathology | Gliosis - metabolism | Mice, Inbred C57BL | Sandhoff Disease - genetics | Genetic Vectors - genetics | Mice, Knockout | Animals | Microfilament Proteins | Mice | Genetic Therapy - methods | Proteins | Disease | Metabolism | Rodents | Genes | Index Medicus
Journal Article
Movement Disorders Clinical Practice, ISSN 2330-1619, 09/2015, Volume 2, Issue 3, pp. 289 - 290
Late‐onset Tay‐Sachs disease ( LOTS ) is a rare autosomal‐recessive genetic disorder caused by insufficient activity of the lysosomal enzyme,... 
2 | Tay‐Sachs disease | gangliosidosis | stutter | G | hexosaminidase A | M | dysarthria | Speech disorders
Journal Article
Journal Article
Clinica Chimica Acta, ISSN 0009-8981, 1984, Volume 143, Issue 2, pp. 73 - 89
Journal Article