Until now, the rare disease, X-ALD, was untreatable. However, recently a model of the chromosome has been identified, which can help improve the scenario.
FREMONT, CA: Recently, the IDIBELL Neurometabolic Disease group, by collaborating internationally, has recognized a model of chromosome X-linked adrenoleukodystrophy (X-ALD) in the earthworm C. elegans. This is a very rare nervous system disorder with no treatment available until now.
This model allows facilitating and decreasing the cost of the research, which includes studying the mechanisms and the probable pharmacological targets for the neuronal modifications of this disease.
A useful genetic tool
The adrenoleukodystrophy’s model in C. elegans is considered a valuable genetic tool as it empowers the researchers to study the mechanisms that are involved in the disease and find the pharmacological trends quicker in comparison to other animal models, like mice, which is even more intricate and involve an expensive and process, which is economically costly.
The researchers got their first results, which, in spite of being preliminary, suggest that glial cells, which are present in the brain together with neurons, will be the first one to be responsible for the neurological modifications associated with the disease.
The latest animal model has helped the researchers in confirming that the oxidative stress caused by mitochondria is the significant reason for neural damage in adrenoleukodystrophy.
C.elegans, neurons, and genes like humans
C.elegans are broadly utilized in biomedical research because, in spite of being simple and small, it possesses the most intricate animal structures and share the majority of the metabolic pathways with humans. Moreover, 40 percent of its genome is homologous to human genes and possess a representation of all the neural populations in the brain.
This organism’s genetic manipulation for developing transgenic models to the study of the disease, like X ALD, is quicker than with other animal models, like rats or mice. Overall, considering characteristics, such as numerous reproductions, body transparency, and the low cost, makes the C.elegans a suitable tool for lab use.