The Catalytic Reaction Mechanism of the β-Galactocerebrosidase Enzyme Deficient in Krabbe Disease
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DOI: 10.1021/acscatal.0c02609
Abstract: Krabbe disease is a neurodegenerative disorder related to misfunction of β-galactocerebrosidase (GALC), a glycosidase that cata-lyzes the cleavage of β-galactosidic bonds in glycosphingolipids. Her... read more here.
Keywords: krabbe; galactocerebrosidase; catalytic reaction; krabbe disease ... See more keywords
Neuron-specific ablation of the Krabbe disease gene galactosylceramidase in mice results in neurodegeneration
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DOI: 10.1371/journal.pbio.3001661
Abstract: Krabbe disease is caused by a deficiency of the lysosomal galactosylceramidase (GALC) enzyme, which results in the accumulation of galactosylceramide (GalCer) and psychosine. In Krabbe disease, the brunt of demyelination and neurodegeneration is believed to… read more here.
Keywords: galactosylceramidase; krabbe disease; neuron specific; krabbe ... See more keywords