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dc.contributor.authorMarchetti, Desirèe Padilhapt_BR
dc.contributor.authorCoelho, Daniella de Mourapt_BR
dc.contributor.authorCoitinho, Adriana Simonpt_BR
dc.contributor.authorGuzzo, Edson Fernando Müllerpt_BR
dc.contributor.authorPadilha, Rafael Bremmpt_BR
dc.contributor.authorRosa, Gabriel de Limapt_BR
dc.contributor.authorVargas, Carmen Reglapt_BR
dc.date.accessioned2021-04-08T04:17:50Zpt_BR
dc.date.issued2020pt_BR
dc.identifier.issn2357-9730pt_BR
dc.identifier.urihttp://hdl.handle.net/10183/219624pt_BR
dc.description.abstractIntroduction: 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
dc.format.mimetypeapplication/pdfpt_BR
dc.language.isoengpt_BR
dc.relation.ispartofClinical and biomedical research. Porto Alegre. Vol. 40, n. 3 (2020), p. 161-166pt_BR
dc.rightsOpen Accessen
dc.subjectX-linked adrenoleukodystrophyen
dc.subjectAdrenoleucodistrofiapt_BR
dc.subjectÁcidos graxospt_BR
dc.subjectC26:0en
dc.subjectWistar ratsen
dc.subjectComportamento animalpt_BR
dc.subjectOpen fielden
dc.titleIncreased levels of hexacosanoic acid in the brain of Wistar rats : a behavioral studypt_BR
dc.typeArtigo de periódicopt_BR
dc.identifier.nrb001123527pt_BR
dc.type.originNacionalpt_BR


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