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- Publications
- Influence
Myelination in the Absence of Galactocerebroside and Sulfatide: Normal Structure with Abnormal Function and Regional Instability
- T. Coetzee, N. Fujita, +5 authors B. Popko
- Biology, Medicine
- Cell
- 26 July 1996
The vertebrate nervous system is characterized by ensheathment of axons with myelin, a multilamellar membrane greatly enriched in the galactolipid galactocerebroside (GalC) and its sulfated… Expand
Mouse models of Tay–Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism
- K. Sango, Shoji Yamanaka, +8 authors R. Proia
- Biology, Medicine
- Nature Genetics
- 1 October 1995
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 defective… Expand
Paranodal junction formation and spermatogenesis require sulfoglycolipids
- K. Honke, Y. Hirahara, +8 authors N. Taniguchi
- Biology, Medicine
- Proceedings of the National Academy of Sciences…
- 26 March 2002
Mammalian sulfoglycolipids comprise two major members, sulfatide (HSO3-3-galactosylceramide) and seminolipid (HSO3-3-monogalactosylalkylacylglycerol). Sulfatide is a major lipid component of the… Expand
TNFα promotes proliferation of oligodendrocyte progenitors and remyelination
- H. Arnett, J. Mason, M. Marino, K. Suzuki, G. Matsushima, J. Ting
- Biology
- Nature Neuroscience
- 1 November 2001
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 a… Expand
PART 16 : LYSOSOMAL DISORDERS Chapter 145 : Niemann-Pick Disease Type C : A Lipid Trafficking Disorder
- M. Patterson, M. Vanier, +5 authors P. Pentchev
- 2007
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 exogenous… Expand
Oligodendrocytes and progenitors become progressively depleted within chronically demyelinated lesions.
- J. L. Mason, A. Toews, +4 authors G. Matsushima
- Biology, Medicine
- The American journal of pathology
- 1 May 2004
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 the… Expand
Consequences of NPC1 and NPC2 loss of function in mammalian neurons.
- S. Walkley, K. Suzuki
- Biology, Medicine
- Biochimica et biophysica acta
- 11 October 2004
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 transmembrane… Expand
A Myelin Galactolipid, Sulfatide, Is Essential for Maintenance of Ion Channels on Myelinated Axon But Not Essential for Initial Cluster Formation
- T. Ishibashi, J. Dupree, +7 authors H. Baba
- Chemistry, Medicine
- The Journal of Neuroscience
- 1 August 2002
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
Functional Genomic Analysis of Remyelination Reveals Importance of Inflammation in Oligodendrocyte Regeneration
- H. Arnett, Y. Wang, G. Matsushima, K. Suzuki, J. Ting
- Biology, Medicine
- The Journal of Neuroscience
- 29 October 2003
Tumor necrosis factor α (TNFα), a proinflammatory cytokine, was shown previously to promote remyelination and oligodendrocyte precursor proliferation in a murine model for demyelination and… Expand
Peripheral macrophage recruitment in cuprizone-induced CNS demyelination despite an intact blood–brain barrier
- E. J. McMahon, K. Suzuki, G. Matsushima
- Biology, Medicine
- Journal of Neuroimmunology
- 1 September 2002
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 brain… Expand