An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

Kumiko I. Claycomb; Kasey M. Johnson; Ernesto R. Bongarzone; Stephen J. Crocker

doi:10.3791/51903

Journal

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Neurociencias

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Un In Vitro Modelo para el Estudio del Celular Fisiopatología en globoides Leucodistrofia Celular

An <em>In Vitro</em> Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

JoVE Revista
Neurociencias

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JoVE Revista Neurociencias

An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy

Un In Vitro Modelo para el Estudio del Celular Fisiopatología en globoides Leucodistrofia Celular

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7,852 Views

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07:45 min

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October 21, 2014

DOI:

10.3791/51903-v

DOI:

10.3791/51903-v

•

07:45 min

October 21, 2014

•

2 Views

Kumiko I. Claycomb¹, Kasey M. Johnson¹, Ernesto R. Bongarzone², Stephen J. Crocker¹

¹Department of Neuroscience,University of Connecticut Health Center, ²Department of Anatomy and Cell Biology,University of Illinois at Chicago

Summary

Globoid cells are a defining pathological feature of Krabbe disease, a leukodystrophy currently lacking an effective long-term therapy. We have developed a cell culture model to study the innate biology and pathogenic potential of activated microglia and their transformation into globoid cells.