Search Results - "DeLucia, Michael W."
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Single-dose intracerebroventricular administration of galactocerebrosidase improves survival in a mouse model of globoid cell leukodystrophy
Published in The FASEB journal (01-08-2007)“…Globoid cell leukodystrophy (GLD), also known as Krabbe disease, is a devastating, degenerative neurological disorder. It is inherited as an autosomal…”
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2
Enzyme replacement therapy results in substantial improvements in early clinical phenotype in a mouse model of globoid cell leukodystrophy
Published in The FASEB journal (01-09-2005)“…ABSTRACTGloboid cell leukodystrophy (GLD) or Krabbe disease is a devastating, degenerative neurological disorder caused by mutations in the…”
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Alzheimer Disease With Amygdala Lewy Bodies: A Distinct Form of α-Synucleinopathy
Published in Journal of neuropathology and experimental neurology (01-07-2006)“…Lewy bodies (LBs) are α-synuclein-immunoreactive neuronal inclusions with a predilection for specific cortical and subcortical regions, including the amygdala…”
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4
Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD)
Published in Neurobiology of disease (01-08-2006)“…The twitcher mouse is a pathologically and enzymatically authentic model of globoid cell leukodystrophy (GLD, Krabbe disease) that has been widely used for the…”
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Hereditary diffuse leukoencephalopathy with spheroids: clinical, pathologic and genetic studies of a new kindred
Published in Acta neuropathologica (01-04-2006)“…Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare autosomal dominant disorder characterized by cerebral white matter degeneration with…”
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α-Synuclein immunoreactivity in neuronal nuclear inclusions and neurites in multiple system atrophy
Published in Neuroscience letters (09-01-2004)“…We examined neuronal and oligodendroglial nuclear inclusions and neurites in the pontine base of multiple system atrophy brains using an antibody to…”
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Argyrophilic Grain Disease in Demented Subjects Presenting Initially With Amnestic Mild Cognitive Impairment
Published in Journal of neuropathology and experimental neurology (01-06-2006)“…A previous autopsy study of patients with amnestic-type mild cognitive impairment (MCI) suggested an overrepresentation of argyrophilic grain disease (AGD). We…”
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Alzheimer Disease With Amygdala Lewy Bodies: A Distinct Form of [alpha]-Synucleinopathy
Published in Journal of neuropathology and experimental neurology (01-07-2006)“…Lewy bodies (LBs) are alpha-synuclein-immunoreactive neuronal inclusions with a predilection for specific cortical and subcortical regions, including the…”
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