Anti-oxidant MitoQ rescue of AWB chemosensory neuron impairment in a C. elegans model of X-linked Adrenoleukodystrophy
| Autor: | Guha, Sanjib, Pujol, Aurora, Dalfo, Esther |
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| Rok vydání: | 2021 |
| Předmět: | |
| Zdroj: | Dipòsit Digital de Documents de la UAB Universitat Autònoma de Barcelona microPublication Biology |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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