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Myelination in the Absence of Galactocerebroside and Sulfatide: Normal Structure with Abnormal Function and Regional Instability
The vertebrate nervous system is characterized by ensheathment of axons with myelin, a multilamellar membrane greatly enriched in the galactolipid galactocerebroside (GalC) and its sulfatedExpand
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Mouse models of Tay–Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism
Tay–Sachs and Sandhoff diseases are clinically similar neurodegenerative disorders. These two sphingolipidoses are characterized by a heritable absence of β–hexosaminidase A resulting in defectiveExpand
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Paranodal junction formation and spermatogenesis require sulfoglycolipids
Mammalian sulfoglycolipids comprise two major members, sulfatide (HSO3-3-galactosylceramide) and seminolipid (HSO3-3-monogalactosylalkylacylglycerol). Sulfatide is a major lipid component of theExpand
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TNFα promotes proliferation of oligodendrocyte progenitors and remyelination
Here we used mice lacking tumor necrosis factor-α (TNFα) and its associated receptors to study a model of demyelination and remyelination in which these events could be carefully controlled using aExpand
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PART 16 : LYSOSOMAL DISORDERS Chapter 145 : Niemann-Pick Disease Type C : A Lipid Trafficking Disorder
1. Niemann-Pick disease type C (NP-C) is an autosomal recessive lipidosis with protean clinical manifestations, distinguished biochemically by a unique error in cellular trafficking of exogenousExpand
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Oligodendrocytes and progenitors become progressively depleted within chronically demyelinated lesions.
To understand mechanisms that may underlie the progression of a demyelinated lesion to a chronic state, we have used the cuprizone model of chronic demyelination. In this study, we investigated theExpand
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Consequences of NPC1 and NPC2 loss of function in mammalian neurons.
Genetic deficiency of NPC1 or NPC2 results in a devastating cholesterol-glycosphingolipidosis of brain and other organs known as Niemann-Pick type C (NPC) disease. While NPC1 is a transmembraneExpand
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A Myelin Galactolipid, Sulfatide, Is Essential for Maintenance of Ion Channels on Myelinated Axon But Not Essential for Initial Cluster Formation
Myelinated axons are divided into four distinct regions: the node of Ranvier, paranode, juxtaparanode, and internode, each of which is characterized by a specific set of axonal proteins.Expand
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Functional Genomic Analysis of Remyelination Reveals Importance of Inflammation in Oligodendrocyte Regeneration
Tumor necrosis factor α (TNFα), a proinflammatory cytokine, was shown previously to promote remyelination and oligodendrocyte precursor proliferation in a murine model for demyelination andExpand
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Peripheral macrophage recruitment in cuprizone-induced CNS demyelination despite an intact blood–brain barrier
The contribution of peripheral macrophage was assessed in cuprizone intoxication, a model of demyelination and remyelination in which the blood-brain barrier remains intact. Flow cytometry of brainExpand
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