Anti-oxidant MitoQ rescue of AWB chemosensory neuron impairment in a C. elegans model of X-linked Adrenoleukodystrophy
Guha, Sanjib (University of Rochester. Department of Anesthesiology & Perioperative Medicine)
Pujol, Aurora 1968- (Institut d'Investigació Biomèdica de Bellvitge)
Dalfo, Esther (Universitat Autònoma de Barcelona. Institut de Neurociències)

Date:	2021
Abstract:	X-linked Adrenoleukodystrophy (X-ALD) is a neurometabolic disorder caused by a defective peroxisomal ABCD1 transporter of very long-chain fatty acids (VLCFAs). We have characterized a nematode model of X-ALD with loss of the pmp-4 gene, the worm orthologue of ABCD1. These mutants recapitulated the key hallmarks of X-ALD and importantly mitochondria targeted antioxidant MitoQ prevented axonal degeneration and locomotor disability. In this study, we further demonstrated that the AWB chemosensory neuron of the pmp-4 mutant worm is defective, both in morphology and function. Interestingly, MitoQ could rescue both the phenotypes. Collectively, our results suggest that C. elegans' chemosensation might provide a novel setting for exploring peroxisomal disease related disorders. ​.
Grants:	Ministerio de Ciencia e Innovación PI1100968 Ministerio de Ciencia e Innovación PC0009/003
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Published in:	microPublication Biology, Vol. 2021 (january 2021) , ISSN 2578-9430

DOI: 10.17912/micropub.biology.000346
PMID: 33474532

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Research literature > UAB research groups literature > Research Centres and Groups (research output) > Health sciences and biosciences > Institut de Neurociències (INc)
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Articles > Published articles