Laboratório de Doenças Neurometabólicas, Laboratório de Neurologia Experimental, Programa de Pós-Graduação Em Ciências da Saúde, Universidade Do Extremo Sul Catarinense, Criciúma, SC, Brasil.
Translational Psychiatry Program, Department of Psychiatry and Behavioral Sciences, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth), Houston, TX, USA.
Metab Brain Dis. 2022 Jun;37(5):1585-1596. doi: 10.1007/s11011-022-00969-8. Epub 2022 Apr 8.
Maple Syrup Urine Disease (MSUD) is caused by the deficiency in the activity of the branched-chain α-ketoacid dehydrogenase complex (BCKDC), resulting in the accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine, and valine, and their respective branched-chain α-keto acids. Patients with MSUD are at high risk of developing chronic neuropsychiatric disorders; however, the pathophysiology of brain damage in these patients remains unclear. We hypothesize that MSUD can cause depressive symptoms in patients. To test our hypothesis, Wistar rats were submitted to the BCAA and tianeptine (antidepressant) administration for 21 days, starting seven days postnatal. Depression-like symptoms were assessed by testing for anhedonia and forced swimming after treatments. After the last test, the brain structures were dissected for the evaluation of neutrophins. We demonstrate that chronic BCAA administration induced depressive-like behavior, increased BDNF levels, and decreased NGF levels, suggesting a relationship between BCAA toxicity and brain damage, as observed in patients with MSUD. However, the administration of tianeptine was effective in preventing behavioral changes and restoring neurotrophins levels.
枫糖尿症(MSUD)是由于支链α-酮酸脱氢酶复合物(BCKDC)活性缺乏引起的,导致支链氨基酸(BCAA)亮氨酸、异亮氨酸和缬氨酸及其各自的支链α-酮酸积累。MSUD 患者有发生慢性神经精神障碍的高风险;然而,这些患者脑损伤的病理生理学仍不清楚。我们假设 MSUD 可导致患者出现抑郁症状。为了验证我们的假设,我们对 Wistar 大鼠进行了 21 天的支链氨基酸和噻奈普汀(抗抑郁药)给药,从出生后第 7 天开始。在治疗后通过测试快感缺失和强迫游泳来评估抑郁样症状。最后一次测试后,对大脑结构进行解剖以评估中性粒细胞。我们证明慢性支链氨基酸给药可诱导抑郁样行为,增加 BDNF 水平,降低 NGF 水平,表明支链氨基酸毒性与脑损伤之间存在关系,如 MSUD 患者所见。然而,噻奈普汀的给药可有效预防行为改变并恢复神经营养因子水平。