A spontaneously immortalized Schwann cell line to study the molecular aspects of metachromatic leukodystrophy

Saravanan, Konda Mani; Büssow, Heinrich; Weiler, Nina; Gieselmann, Volkmar; Franken, Sebastian

doi:10.1016/j.jneumeth.2006.11.009

Cited by 12 publications

(6 citation statements)

References 48 publications

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“…In the absence of a significant reduction in the number of mature oligodendrocytes, this might indicate the inhibition of signaling processes by sulfatide in fully differentiated oligodendrocytes [73]. MAL mRNA is also reduced in ASA-deficient Schwann cells upon treatment with sulfatide, and this effect can be reversed by the additional endocytosis of ASA [95]. In kidney cells, sulfatide storage is accompanied by a mislocalization of MAL, which switched from the plasma membrane to a late endosomal/lysosomal compartment [65].…”

Section: Pathology Of Sulfatide Storagementioning

confidence: 99%

The Role and Metabolism of Sulfatide in the Nervous System

2008

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3-O-sulfogalactosylceramide or sulfatide is a major component of the myelin sheath in the central and peripheral nervous system. The examination of mice deficient in the sulfatide-synthesizing enzyme, cerebroside sulfotransferase, provided new insight into the role of sulfatide in the differentiation of myelinating cells, formation of the paranodal junction, and myelin maintenance. Although in general regarded as a marker for oligodendrocytes and Schwann cells, sulfatide is also present in astrocytes and neurons. The relatively low amount of sulfatide in neurons can dramatically increase in the absence of the sulfatide-degrading enzyme, arylsulfatase A, as in metachromatic leukodystrophy. Recent advance in the understanding of this disease comes from studies on new transgenic mouse models. Significant changes in sulfatide levels have also been observed more recently in Alzheimer's disease and other diseases, suggesting that sulfatide might be involved in the pathogenesis of these diseases as well. This review summarizes recent studies on the physiological and pathophysiological role of sulfatide using transgenic mice deficient in its synthesis or degradation.

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Section: Pathology Of Sulfatide Storagementioning

confidence: 99%

The Role and Metabolism of Sulfatide in the Nervous System

2008

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“…The immortalized Schwann cell lines have proven useful in the study of the human sphingolipidoses, since mouse models for these diseases can be used to spontaneously derive mouse Schwann cell lines which can be useful in the understanding of the molecular basis for these diseases. For example, spontaneous mouse Schwann cell lines have been derived from appropriate mouse models for Niemann-Pick disease type c [73], globoid cell leukodystrophy [74], Sandhoff's disease [75] and metachromatic leukodystrophy [76]. A detailed description of the methods used to establish immortalized Schwann cells from murine disease models has been published [77].…”

Section: Tsc Rat Schwann Cell Line: Culture Conditionsmentioning

confidence: 99%

Glial Cell Lines: An Overview

Vries

Boullerne

2010

Neurochem Res

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The importance and essential functions of glial cells in the nervous system are now beginning to be understood and appreciated. Glial cell lines have been instrumental in the elucidation of many of these properties. In this Overview, the origin and properties of most of the existing cell lines for the major glial types: oligodendroglia, astroglia, microglia and Schwann cells, are documented. Particular emphasis is given to the culture conditions for each cell line and the degree to which the line can differentiate in vitro and in vivo. The major molecular markers for each glial cell lines are indicated. Finally, methods by which the glial cell lines have been developed are noted and the future directions of glial cell line research are discussed.

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“…Rat monoclonal antibody GL2A7 specifically reacts with the mouse lysosomal protein LAMP‐2 by immunoblot (Saravanan et al,2004) and by immunohistochemistry (Gondre‐Lewis et al, 2004; Saravanan et al,2007).…”

Section: Methodsmentioning

confidence: 99%

“Late” macroendosomes and acidic endosomes in vertebrate motor nerve terminals

Stewart¹,

Teng²,

Wilkinson³

2012

J of Comparative Neurology

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Activity at the vertebrate nerve—muscle synapse creates large macroendosomes (MEs) via bulk membrane infolding. Visualized with the endocytic probe FM1-43, most (94%) of the ~25 MEs/terminal created by brief (30-Hz, 18-second) stimulation dissipate rapidly (~1 minute) into vesicles. Others, however, remain for hours. Here we study these “ late” MEs by using 4D live imaging over a period of ~1 hour after stimulation. We find that some (51/398 or 13%) disappear spontaneously via exocytosis, releasing their contents into the extracellular milieu. Others (at least 15/1,960 or 1%) fuse or closely associate with a second class of endosomes that take up acidophilic dyes (acidic endosomes [AEs]). AEs are plentiful (~47/terminal) and exist independent of stimulation. Unlike MEs, which exhibit Brownian motion, AEs exhibit directed motion (average, 83 nm/sec) on microtubules within and among terminal boutons. AEs populate the axon as well, where movement is predominantly retrograde. They share biochemical and immunohistochemical markers (e.g., lysosomal-associated membrane protein [LAMP-1]) with lysosomes. Fusion/association of MEs with AEs suggests a sorting/degradation pathway in nerve terminals wherein the role of AEs is similar to that of lysosomes. Based on our data, we propose that MEs serve as sorting endosomes. Thus their contents, which include plasma membrane proteins, vesicle proteins, and extracellular levels of Ca2+, can be targeted either toward the reformation and budding of synaptic vesicles, toward secretion via exocytosis, or toward a degradation process that utilizes AEs either for lysis within the terminal or for transport toward the cell body.

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A spontaneously immortalized Schwann cell line to study the molecular aspects of metachromatic leukodystrophy

Cited by 12 publications

References 48 publications

The Role and Metabolism of Sulfatide in the Nervous System

The Role and Metabolism of Sulfatide in the Nervous System

Glial Cell Lines: An Overview

“Late” macroendosomes and acidic endosomes in vertebrate motor nerve terminals

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