Abnormal activation of MAPKs pathways and inhibition of autophagy in a group of patients with Zellweger spectrum disorders and X-linked adrenoleukodystrophy

Abnormal activation of MAPKs pathways and inhibition of autophagy in a group of patients with Zellweger spectrum disorders and X-linked adrenoleukodystrophy

Abstract Background Zellweger spectrum disorders (ZSD) and X-linked adrenoleukodystrophy (X-ALD) are inherited metabolic diseases characterized by dysfunction of peroxisomes, that are essential for lipid metabolism and redox balance. Oxidative stress has been reported to have a significant role in t...

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Bibliographic Details
Main Authors: Vincenza Gragnaniello, Daniela Gueraldi, Andrea Puma, Anna Commone, Chiara Cazzorla, Christian Loro, Elena Porcù, Maria Stornaiuolo, Paolo Miglioranza, Leonardo Salviati, Ronald J. A. Wanders, Alberto Burlina
Format: Article
Language:English
Published: BMC 2023-11-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Peroxisomal disorders
Zellweger spectrum disorders
X-linked adrenoleukodystrophy
Oxidative stress
Autophagy
Mitogen-activated protein kinases (MAPKs)
Online Access:https://doi.org/10.1186/s13023-023-02940-x

Internet

https://doi.org/10.1186/s13023-023-02940-x

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