Recent Publications on Sandhoff Disease: |  |
Sphingosine kinase 1/S1P receptor signaling axis controls glial proliferation in mice with Sandhoff disease.
Sphingosine-1-phosphate (S1P) is a lipid-signaling molecule produced by...
Direct Link | 5th August, 2008
| Genetics of Development and Disease Branch, National Institute of Diabetes
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Peripheral blood mononuclear cell infiltration and neuroinflammation in the HexB(-/-) mouse model of neurodegeneration.
Myeloid-derived immune cells, including microglia, macrophages and...
Direct Link | 29th July, 2008
| Department of Neurobiology & Anatomy, School of Medicine & Dentistry,
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N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice.
Sandhoff disease involves the CNS accumulation of ganglioside GM2 and...
Direct Link | 29th July, 2008
| Biology Department, Boston College, 140 Commonwealth Avenue, Chestnut
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Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease.
Mouse models of lysosomal storage diseases, including Sandhoff disease,...
Direct Link | 10th July, 2008
| Behavioral Core Facility, Dominick S. Purpura Department of Neuroscience,
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Proton MRS of a child with Sandhoff disease reveals elevated brain hexosamine.
Sandhoff disease (gangliosidosis type 0) is a lysosomal storage disorder...
Direct Link | 31st May, 2008
| Abteilung Neuropadiatrie, Department of Pediatric Neurology, Klinikum
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Imaging MALDI mass spectrometry using an oscillating capillary nebulizer matrix coating system and its application to analysis of lipids in brain from a mouse model of Tay-Sachs/Sandhoff disease.
The quality of tissue imaging by matrix-assisted laser...
Direct Link | 21st May, 2008
| School of Chemistry and Biochemistry, The Parker H. Petit Institute for
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Simultaneous quantification of GM1 and GM2 gangliosides by isotope dilution tandem mass spectrometry.
OBJECTIVES: Gangliosides (GGs) are considered as diagnostic biomarkers and...
Direct Link | 20th May, 2008
| Department of Laboratory Medicine, Children's National Medical Center, 111
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Involvement of Retinal Neurons and Pigment Epithelial Cells in a Murine Model of Sandhoff Disease.
Background/Aims: To investigate the effects of deficient degradation of...
Direct Link | 26th April, 2008
| Department of Developmental Morphology, Tokyo Metropolitan Institute for
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Neuronal and glial accumulation of alpha- and beta-synucleins in human lipidoses.
A number of the lysosomal storage diseases that have now been...
Direct Link | 20th February, 2008
| Department of Psychiatry, Yokohama City University School of Medicine, 3-9
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| Substrate deprivation therapy: a new hope for patients suffering from neuronopathic forms of inherited lysosomal storage diseases.
| 29th January, 2008
| Department of Molecular Biology, University of Gdansk, Kladki 24, 80-822
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Increased lung surfactant phosphatidylcholine in patients affected by lysosomal storage diseases.
Sandhoff disease, Gaucher disease type I and sialidosis type I are...
Direct Link | 9th January, 2008
| Department of Neurological and Behavioural Sciences, Section of
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Lending a helping hand, screening chemical libraries for compounds that enhance beta-hexosaminidase A activity in GM2 gangliosidosis cells.
Enzyme enhancement therapy is an emerging therapeutic approach that has...
Direct Link | 19th December, 2007
| Research Institute, SickKids, Toronto, Ontario, Canada.
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Peripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.
BACKGROUND: Sandhoff disease is an inherited lysosomal storage disease...
Direct Link | 14th November, 2007
| Biology Department, Boston College, 140 Commonwealth Avenue, Chestnut
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Intraperitoneal inoculation of Sandhoff mouse neonates with an HIV-1 based lentiviral vector exacerbates the attendant neuroinflammation and disease phenotype.
We aimed to evaluate the efficacy of VSV-G pseudotyped, defective HIV-1...
Direct Link | 20th October, 2007
| Eastman Dental Center, University of Rochester School of Medicine and
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| The cranial base in craniofacial development: a gene therapy study.
| 18th October, 2007
| Departments of Dentistry, School of Medicine and Dentistry, University of
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