| dc.creator | Marchetti, Desiree | |
| dc.creator | Coelho, Daniella de M | |
| dc.creator | Coitinho, Adriana S | |
| dc.creator | Guzzo, Edson F M | |
| dc.creator | Padilha, Rafael B | |
| dc.creator | Rosa, Gabriel de L | |
| dc.creator | Vargas, Carmen R | |
| dc.date | 2021-03-11 | |
| dc.date.accessioned | 2022-10-04T21:04:36Z | |
| dc.date.available | 2022-10-04T21:04:36Z | |
| dc.identifier | https://seer.ufrgs.br/index.php/hcpa/article/view/103339 | |
| dc.identifier.uri | http://repositorioslatinoamericanos.uchile.cl/handle/2250/3865710 | |
| dc.description | 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 (C26:0) accumulation represents a hallmark in the pathogenesis of this disease. In this study, we examined whether an overload of C26:0 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 C26:0 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 C26:0 administration presented fewer crossings and rearing episodes and a higher latency to move 45 minutes after C26:0 injection. The present work yields experimental evidence that C26:0, 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 C26:0 accumulation and locomotor impairment in patients with X-ALD. | en-US |
| dc.format | application/pdf | |
| dc.language | eng | |
| dc.publisher | HCPA/FAMED/UFRGS | pt-BR |
| dc.relation | https://seer.ufrgs.br/index.php/hcpa/article/view/103339/pdf | |
| dc.rights | Copyright (c) 2021 Clinical and Biomedical Research | pt-BR |
| dc.source | Clinical & Biomedical Research; Vol. 40 No. 3 (2020) | en-US |
| dc.source | Clinical and Biomedical Research; v. 40 n. 3 (2020) | pt-BR |
| dc.source | 2357-9730 | |
| dc.subject | X-linked adrenoleukodystrophy | en-US |
| dc.subject | C26 | en-US |
| dc.subject | Wistar rats | en-US |
| dc.subject | open field. | en-US |
| dc.title | Increased levels of hexacosanoic acid in the brain of Wistar rats: a behavioral study | en-US |
| dc.type | info:eu-repo/semantics/article | |
| dc.type | info:eu-repo/semantics/publishedVersion | |
| dc.type | Peer-reviewed Article | en-US |
| dc.type | Avaliado por Pares | pt-BR |
