Peroxisomal Multifunctional Protein 2 Deficiency Perturbs Lipid Homeostasis in the Retina and Causes Visual Dysfunction in Mice
Patients lacking multifunctional protein 2 (MFP2), the central enzyme of the peroxisomal β-oxidation pathway, develop retinopathy. This pathway is involved in the metabolism of very long chain (VLCFAs) and polyunsaturated (PUFAs) fatty acids, which are enriched in the photoreceptor outer segments (P...
Main Authors: | Yannick Das, Daniëlle Swinkels, Sai Kocherlakota, Stefan Vinckier, Frédéric M. Vaz, Eric Wever, Antoine H. C. van Kampen, Bokkyoo Jun, Khanh V. Do, Lieve Moons, Nicolas G. Bazan, Paul P. Van Veldhoven, Myriam Baes |
---|---|
Format: | Article |
Language: | English |
Published: |
Frontiers Media S.A.
2021-02-01
|
Series: | Frontiers in Cell and Developmental Biology |
Subjects: | |
Online Access: | https://www.frontiersin.org/articles/10.3389/fcell.2021.632930/full |
Similar Items
-
Peroxisomal Disorders and Their Mouse Models Point to Essential Roles of Peroxisomes for Retinal Integrity
by: Yannick Das, et al.
Published: (2021-04-01) -
Peroxisome deficient invertebrate and vertebrate animal models
by: Paul P. Van Veldhoven, et al.
Published: (2013-11-01) -
Early-onset Purkinje cell dysfunction underlies cerebellar ataxia in peroxisomal multifunctional protein-2 deficiency
by: Stephanie De Munter, et al.
Published: (2016-10-01) -
Lipidomic analysis of fibroblasts from Zellweger spectrum disorder patients identifies disease-specific phospholipid ratios[S]
by: Katharina Herzog, et al.
Published: (2016-08-01) -
De novo peroxisome biogenesis revisited
by: Marten Veenhuis, et al.
Published: (2014-04-01)