Recent Publications on Galactosylceramidase: |  |
Peripheral neuropathy in the twitcher mouse: accumulation of extracellular matrix in the endoneurium and aberrant expression of ion channels.
Globoid cell leukodystrophy (GLD; Krabbe's disease), caused by a genetic...
Direct Link | 25th September, 2008
| Department of Pathology and Laboratory Medicine, University of North
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Clinical and immunopathologic alterations in rhesus macaques affected with globoid cell leukodystrophy.
Globoid cell leukodystrophy, or Krabbe's disease, is a severe disorder of...
Direct Link | 3rd April, 2008
| DVM, PhD, Division of Comparative Pathology, Tulane National Primate
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Central nervous system-directed AAV2/5-mediated gene therapy synergizes with bone marrow transplantation in the murine model of globoid-cell leukodystrophy.
Globoid-cell leukodystrophy (GLD) is a rapidly progressing inherited...
Direct Link | 1st April, 2008
| Department of Internal Medicine, Washington University School of Medicine,
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Cellular uptake and lysosomal delivery of galactocerebrosidase tagged with the HIV Tat protein transduction domain.
A number of studies have shown that a short peptide, the protein...
Direct Link | 15th February, 2008
| Department of Medicine, Louisiana State University Health Sciences Center,
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Autonomic denervation of lymphoid organs leads to epigenetic immune atrophy in a mouse model of Krabbe disease.
Lysosomal beta-galactosylceramidase deficiency results in demyelination...
Direct Link | 23rd January, 2008
| Department of Anatomy, College of Medicine, University of Illinois at
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Single-dose intracerebroventricular administration of galactocerebrosidase improves survival in a mouse model of globoid cell leukodystrophy.
Globoid cell leukodystrophy (GLD), also known as Krabbe disease, is a...
Direct Link | 6th November, 2007
| Mayo Clinic College of Medicine, 4500 San Pablo Rd., Jacksonville, Florida
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Psychosine-induced apoptosis and cytokine activation in immune peripheral cells of Krabbe patients.
Globoid cell leukodystrophy or Krabbe disease (KD), is a hereditary...
Direct Link | 28th September, 2007
| Department of Neurological and Behavioural Sciences, University of Siena,
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Molecular beacon genotyping for globoid cell leukodystrophy from hair roots in the twitcher mouse and rhesus macaque.
Rapid and accurate genotype determination is ideal for the maintenance of...
Direct Link | 19th August, 2007
| Department of Biology, University of New Orleans, 2000 Lakeshore Drive,
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A single mutation in the GALC gene is responsible for the majority of late onset Krabbe disease patients in the Catania (Sicily, Italy) region.
A high proportion of patients with late onset forms of Krabbe disease is...
Direct Link | 25th July, 2007
| Center for Medical Genetics, Universitair Ziekenhuis Brussel, Brussels,
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Diagnosis of Krabbe disease by use of a natural substrate.
This chapter describes in detail a practical procedure for the preparation...
Direct Link | 31st January, 2007
| Genetic-Metabolic Laboratory, Department of Pediatric Laboratory Medicine,
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Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD).
The twitcher mouse is a pathologically and enzymatically authentic model...
Direct Link | 9th December, 2006
| Department of Pharmacology, Mayo Clinic College of Medicine, Jacksonville,
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Undifferentiated mouse mesenchymal stem cells spontaneously express neural and stem cell markers Oct-4 and Rex-1.
BACKGROUND: Previous adult stem cells studies have provided evidence that...
Direct Link | 25th October, 2006
| Department of Neurology, Centre for Immunology, St Vincent's Hospital,
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Six novel mutations detected in the GALC gene in 17 Japanese patients with Krabbe disease, and new genotype-phenotype correlation.
Krabbe disease is an autosomal recessive leukodystrophy. It is...
Direct Link | 29th September, 2006
| Department of Pediatrics, Osaka University Graduate School of Medicine,
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Design and optimization of lentiviral vectors for transfer of GALC expression in Twitcher brain.
BACKGROUND: Demyelination in globoid cell leukodystrophy (GLD) is due to a...
Direct Link | 22nd September, 2006
| Telethon Institute for Gene Therapy, San Raffaele Scientific Institute,
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Intrinsic resistance of neural stem cells to toxic metabolites may make them well suited for cell non-autonomous disorders: evidence from a mouse model of Krabbe leukodystrophy.
While transplanted neural stem cells (NSCs) have been shown to hold...
Direct Link | 12th August, 2006
| Department of Animal Science, Faculty of Veterinary Science, University of
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