Increased levels of hexacosanoic acid in the brain of Winstar rats: a behavioral study
Clin. biomed. res
;
40(3): 161-166, 2020. graf
Article
Dans Anglais
| LILACS
| ID: biblio-1248278
ABSTRACT
Introduction:
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal metabolic disorder associated with mutations in the ATP-binding cassette sub-family D member1 (ABCD1) gene. Practically all male patients with X-ALD develop adrenocortical insufficiency during childhood and progressive myelopathy and peripheral neuropathy in adulthood. However, some male patients develop a fatal cerebral demyelinating disease named cerebral adrenoleukodystrophy. Although the exact mechanisms underlying brain damage in X-ALD are still poorly elucidated, it is known that hexacosanoic acid (C260) accumulation represents a hallmark in the pathogenesis of this disease. In this study, we examined whether an overload of C260 injected in Wistar rats was capable of causing behavioral changes in these animals.Methods:
Egg lecithin in ethanol was dried under a nitrogen stream and mixed with C260 methyl ester. Male Wistar rats at 2-3 weeks of age were obtained from Universidade Federal do Rio Grande do Sul (UFRGS), divided into 8 groups, and submitted to an open field test. We then analyzed line crossings (locomotion and exploration), rearing (orienting and investigatory responses), grooming (anxiety manifestation), and latency to move for each animal.Results:
Animals subjected to C260 administration presented fewer crossings and rearing episodes and a higher latency to move 45 minutes after C260 injection. The present work yields experimental evidence that C260, the main accumulated metabolite in X-ALD, can cause behavioral alterations in rats such as the impairment of locomotion and exploratory capabilities, as well as a reduction in orienting and investigatory responses.Conclusion:
Although our results are preliminary, they are extremely important for future studies that investigate C260 accumulation and locomotor impairment in patients with X-ALD. (AU)
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Comportement
/
Rat Wistar
Limites du sujet:
Animaux
langue:
Anglais
Texte intégral:
Clin. biomed. res
Thème du journal:
Médicament
Année:
2020
Type:
Article
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
Hospital de Clínicas de Porto Alegre/BR
/
Universidade Federal do Rio Grande do Sul/BR
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