Peroxisomal multifunctional protein-2 deficiency causes neuroinflammation and degeneration of Purkinje cells independent of very long chain fatty acid accumulation

Peroxisomal multifunctional protein-2 deficiency causes neuroinflammation and degeneration of Purkinje cells independent of very long chain fatty acid accumulation

Although peroxisome biogenesis and β-oxidation disorders are well known for their neurodevelopmental defects, patients with these disorders are increasingly diagnosed with neurodegenerative pathologies. In order to investigate the cellular mechanisms of neurodegeneration in these patients, we develo...

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Bibliographic Details
Main Authors: Simon Verheijden, Astrid Bottelbergs, Olga Krysko, Dmitri V. Krysko, Lien Beckers, Stephanie De Munter, Paul P. Van Veldhoven, Sabine Wyns, Wim Kulik, Klaus-Armin Nave, Matt S. Ramer, Peter Carmeliet, Celia M. Kassmann, Myriam Baes
Format: Article
Language:English
Published: Elsevier 2013-10-01
Series:Neurobiology of Disease
Subjects:
X-linked adrenoleukodystrophy
D-bifunctional protein deficiency
Peroxisomal β-oxidation
Multifunctional protein-2
HSD17B4
Mouse model
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996113001733

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http://www.sciencedirect.com/science/article/pii/S0969996113001733

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