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"This model of adrenoleukodystrophy in C. elegans is a very valuable genetic tool that will allow us to study the mechanisms involved in the disease and to find pharmacological targets faster than with other animal models, such as mice, which are much more complex and involve a costly and economically expensive process," says Esther Dalfó, who now is leading the C elegans models of diseases group between INC-UAB and the UVic-UCC.
The research team has obtained its first results, which, despite being preliminary, suggest that glial cells, which are in the brain together with neurons, would be the first responsible for the neurological alterations associated with the disease.
This new animal model has helped us to confirm that oxidative stress caused by mitochondria (the energy-producing organelles of cells) is the major cause of neuronal damage in adrenoleukodystrophy, and this mechanism of damage is conserved from the worm to the patient. The data point to new therapeutic pathways, such as mitochondrial antioxidants in peroxisome disease." Aurora Pujol ICREA professor at IDIBELL C. elegans , neurons and genes like humans
The earthworm C. elegans is widely used in biomedical research because, despite being simple and small (measuring only 1 mm), it has most of the most complex animal structures and share most of the metabolic pathways with us. Besides, 40% of its genome is homologous to our genes and has a representation of all the neural populations in the human brain.
The genetic manipulation of this organism to create transgenic models to the study of the disease, such as X ALD, is faster than with other animal models such as rats or mice. Overall, taking into account characteristics like body transparency, numerous reproductions -up to 300 descendants- and the low cost, make the C. elegans an ideal tool for lab use. Source:
IDIBELL-Bellvitge Biomedical Research Institute Journal reference:
Coppa, A., et al. (2020) The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophy. Free Radical Biology and Medicine . doi.org/10.1016/j.freeradbiomed.2020.01.177 .
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