دورية أكاديمية
Branched-chain amino acids (BCAA) administration increases autophagy and the autophagic pathway in brain tissue of rats submitted to a Maple Syrup Urine Disease (MSUD) protocol.
| العنوان: | Branched-chain amino acids (BCAA) administration increases autophagy and the autophagic pathway in brain tissue of rats submitted to a Maple Syrup Urine Disease (MSUD) protocol. |
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| المؤلفون: | Fermo KT; Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brasil., da Silva Lemos I; Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brasil., Farias HR; Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brasil., Rosso MP; Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brasil., Effting PS; Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brasil., Leipnitz G; Departamento de Bioquímica, Universidade Federal Do Rio Grande Sul, Porto Alegre, RS, 90035-003, Brasil., Streck EL; Laboratório de Doenças Neurometabólicas, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, 88806-000, Brasil. emiliostreck@gmail.com. |
| المصدر: | Metabolic brain disease [Metab Brain Dis] 2023 Jan; Vol. 38 (1), pp. 287-293. Date of Electronic Publication: 2022 Oct 28. |
| نوع المنشور: | Journal Article; Research Support, Non-U.S. Gov't |
| اللغة: | English |
| بيانات الدورية: | Publisher: Springer Country of Publication: United States NLM ID: 8610370 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1573-7365 (Electronic) Linking ISSN: 08857490 NLM ISO Abbreviation: Metab Brain Dis Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: 2005- : Amsterdam : Springer Original Publication: New York : Plenum, c1986- |
| مواضيع طبية MeSH: | Maple Syrup Urine Disease*/metabolism, Rats ; Animals ; Amino Acids, Branched-Chain/metabolism ; Rats, Wistar ; Disease Models, Animal ; Brain/metabolism ; Keto Acids ; Autophagy |
| مستخلص: | Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism (EIM) biochemically characterized by the tissue accumulation of branched-chain amino acids (BCAA) and their branched-chain alpha-keto acids. The mechanisms by which BCAA and their branched-chain alpha-keto acids lead to the neurological damage observed in MSUD are poorly understood. Mounting evidence has demonstrated that BCAA induce the overproduction of reactive oxygen species, which may modulate several important signaling pathways necessary for cellular homeostasis maintenance, such as autophagy. Taking this into account, we evaluated the effects of BCAA on the autophagic pathway in brain structures of rats submitted to the administration of these amino acids (animal model of MSUD). Our findings showed that BCAA significantly increased the levels of Beclin-1, ATG7, and ATG5 in the cerebral cortex of rats. In addition, BCAA augmented ATG12 levels in the striatum and ATG5 and LC3 I-II in the hippocampus. Therefore, our work demonstrates that the administration of BCAA increases autophagy and autophagic cell death, possibly mediated by the elevated levels of reactive species generated by BCAA. (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.) |
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| فهرسة مساهمة: | Keywords: Autophagy; Branched-chain amino acids; Maple syrup urine disease; Oxidative stress |
| المشرفين على المادة: | 0 (Amino Acids, Branched-Chain) 0 (Keto Acids) |
| تواريخ الأحداث: | Date Created: 20221028 Date Completed: 20230113 Latest Revision: 20230202 |
| رمز التحديث: | 20230202 |
| DOI: | 10.1007/s11011-022-01109-y |
| PMID: | 36305998 |
| قاعدة البيانات: | MEDLINE |
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