Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease
| العنوان: | Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease |
|---|---|
| المؤلفون: | Daniel Pens Gelain, Giselli Scaini, Gustavo C. Ferreira, Camila B. Furlanetto, João Paulo Almeida dos Santos, Matheus Augusto de Bittencourt Pasquali, Patrícia F. Schuck, José Cláudio Fonseca Moreira, Thais Ceresér Vilela, Emilio L. Streck |
| المصدر: | Metabolic Brain Disease. 32:115-122 |
| بيانات النشر: | Springer Science and Business Media LLC, 2016. |
| سنة النشر: | 2016 |
| مصطلحات موضوعية: | Male, 0301 basic medicine, medicine.medical_specialty, bcl-X Protein, Apoptosis, Hippocampus, Biochemistry, 03 medical and health sciences, Cellular and Molecular Neuroscience, 0302 clinical medicine, Bcl-2-associated X protein, Maple Syrup Urine Disease, Valine, Internal medicine, medicine, Animals, Rats, Wistar, bcl-2-Associated X Protein, Cerebral Cortex, chemistry.chemical_classification, Caspase 8, biology, Caspase 3, Maple syrup urine disease, medicine.disease, Rats, Amino acid, Disease Models, Animal, 030104 developmental biology, Endocrinology, medicine.anatomical_structure, Proto-Oncogene Proteins c-bcl-2, chemistry, Cerebral cortex, Inborn error of metabolism, biology.protein, Neurology (clinical), Isoleucine, Leucine, Amino Acids, Branched-Chain, 030217 neurology & neurosurgery, Signal Transduction |
| الوصف: | Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of the branched-chain α-keto acid dehydrogenase complex activity. This blockage leads to accumulation of the branched-chain amino acids leucine, isoleucine and valine, as well as their corresponding α-keto acids and α-hydroxy acids. The affected patients present severe neurological symptoms, such as coma and seizures, as well as edema and cerebral atrophy. Considering that the mechanisms of the neurological symptoms presented by MSUD patients are still poorly understood, in this study, protein levels of apoptotic factors are measured, such as Bcl-2, Bcl-xL, Bax, caspase-3 and -8 in hippocampus and cerebral cortex of rats submitted to acute administration of branched-chain amino acids during their development. The results in this study demonstrated that BCAA acute exposure during the early postnatal period did not significantly change Bcl-2, Bcl-xL, Bax and caspase-8 protein levels. However, the Bax/Bcl-2 ratio and procaspase-3 protein levels were decreased in hippocampus. On the other hand, acute administration of BCAA in 30-day-old rats increase in Bax/Bcl-2 ratio followed by an increased caspase-3 activity in cerebral cortex, whereas BCAA induces apoptosis in hippocampus through activation and cleavage of caspase-3 and -8 without changing the Bax/Bcl-2 ratio. In conclusion, the results suggest that apoptosis could be of pivotal importance in the developmental neurotoxic effects of BCAA. In addition, the current studies also suggest that multiple mechanisms may be involved in BCAA-induced apoptosis in the cerebral cortex and hippocampus. |
| تدمد: | 1573-7365 0885-7490 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::615c541b45bc1c02affdd930764d8a25 https://doi.org/10.1007/s11011-016-9892-0 |
| حقوق: | CLOSED |
| رقم الأكسشن: | edsair.doi.dedup.....615c541b45bc1c02affdd930764d8a25 |
| قاعدة البيانات: | OpenAIRE |
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