Laboratory of Neurobiology, Centro de Investigación Príncipe Felipe, Primo-Yufera 3, 46012, Valencia, Spain.
Mol Neurobiol. 2023 Jun;60(6):3071-3085. doi: 10.1007/s12035-023-03264-4. Epub 2023 Feb 15.
Chronic hyperammonemia is a main contributor to the cognitive and motor impairment in patients with hepatic encephalopathy. Sustained hyperammonemia induces the TNFα expression in Purkinje neurons, mediated by NF-κB activation. The aims were the following: (1) to assess if enhanced TrkB activation by BDNF is responsible for enhanced NF-κB activation in Purkinje neurons in hyperammonemic rats, (2) to assess if this is associated with increased content of NF-κB modulated proteins such as TNFα, HMGB1, or glutaminase I, (3) to assess if these changes are due to enhanced activation of the TNFR1-S1PR2-CCR2-BDNF-TrkB pathway, (4) to analyze if increased activation of NF-κB is mediated by the PI3K-AKT pathway. It is shown that, in the cerebellum of hyperammonemic rats, increased BDNF levels enhance TrkB activation in Purkinje neurons leading to activation of PI3K, which enhances phosphorylation of AKT and of IκB, leading to increased nuclear translocation of NF-κB which enhances TNFα, HMGB1, and glutaminase I content. To assess if the changes are due to enhanced activation of the TNFR1-S1PR2-CCR2 pathway, we blocked TNFR1 with R7050, S1PR2 with JTE-013, and CCR2 with RS504393. These changes are reversed by blocking TrkB, PI3K, or the TNFR1-SP1PR2-CCL2-CCR2-BDNF-TrkB pathway at any step. In hyperammonemic rats, increased levels of BDNF enhance TrkB activation in Purkinje neurons, leading to activation of the PI3K-AKT-IκB-NF-κB pathway which increased the content of glutaminase I, HMGB1, and TNFα. Enhanced activation of this TrkB-PI3K-AKT-NF-κB pathway would contribute to impairing the function of Purkinje neurons and motor function in hyperammonemic rats and likely in cirrhotic patients with minimal or clinical hepatic encephalopathy.
慢性高血氨是肝性脑病患者认知和运动障碍的主要原因。持续的高血氨诱导 TNFα 在浦肯野神经元中的表达,这是由 NF-κB 激活介导的。目的如下:(1)评估 BDNF 增强的 TrkB 激活是否负责高氨血症大鼠浦肯野神经元中 NF-κB 的增强激活,(2)评估这是否与 NF-κB 调节蛋白如 TNFα、HMGB1 或谷氨酰胺酶 I 的含量增加有关,(3)评估这些变化是否是由于 TNFR1-S1PR2-CCR2-BDNF-TrkB 途径的增强激活引起的,(4)分析 NF-κB 的增强激活是否由 PI3K-AKT 途径介导。结果表明,在高氨血症大鼠的小脑,BDNF 水平的增加增强了浦肯野神经元中 TrkB 的激活,导致 PI3K 的激活,从而增强了 AKT 和 IκB 的磷酸化,导致 NF-κB 的核易位增加,从而增强了 TNFα、HMGB1 和谷氨酰胺酶 I 的含量。为了评估这些变化是否是由于 TNFR1-S1PR2-CCR2 途径的增强激活引起的,我们用 R7050 阻断 TNFR1,用 JTE-013 阻断 S1PR2,用 RS504393 阻断 CCR2。通过在任何步骤阻断 TrkB、PI3K 或 TNFR1-S1PR2-CCL2-CCR2-BDNF-TrkB 途径,可以逆转这些变化。在高氨血症大鼠中,BDNF 水平的增加增强了浦肯野神经元中 TrkB 的激活,导致 PI3K-AKT-IκB-NF-κB 途径的激活,增加了谷氨酰胺酶 I、HMGB1 和 TNFα 的含量。这种 TrkB-PI3K-AKT-NF-κB 途径的过度激活可能导致高氨血症大鼠浦肯野神经元功能和运动功能受损,并且可能在最小或临床肝性脑病的肝硬化患者中受损。