Wisconsin National Primate Research Center, University of Wisconsin-Madison, Madison, WI, United States of America.
Cellular and Molecular Pathology Graduate Program, University of Wisconsin-Madison, Madison, WI, United States of America.
PLoS One. 2020 Jan 7;15(1):e0226999. doi: 10.1371/journal.pone.0226999. eCollection 2020.
Cardiac dysautonomia is a common nonmotor symptom of Parkinson's disease (PD) associated with loss of sympathetic innervation to the heart and decreased plasma catecholamines. Disease-modifying strategies for PD cardiac neurodegeneration are not available, and biomarkers of target engagement are lacking. Systemic administration of the catecholaminergic neurotoxin 6-hydroxydopamine (6-OHDA) recapitulates PD cardiac dysautonomia pathology. We recently used positron emission tomography (PET) to visualize and quantify cardiac sympathetic innervation, oxidative stress, and inflammation in adult male rhesus macaques (Macaca mulatta; n = 10) challenged with 6-OHDA (50mg/kg; i.v.). Twenty-four hours post-intoxication, the animals were blindly and randomly assigned to receive daily doses of the peroxisome proliferator-activated receptor gamma (PPARγ) agonist pioglitazone (n = 5; 5mg/kg p.o.) or placebo (n = 5). Quantification of PET radioligand uptake showed increased oxidative stress and inflammation one week after 6-OHDA which resolved to baseline levels by twelve weeks, at which time pioglitazone-treated animals showed regionally preserved sympathetic innervation. Here we report post mortem characterization of heart and adrenal tissue in these animals compared to age and sex matched normal controls (n = 5). In the heart, 6-OHDA-treated animals showed a significant loss of sympathetic nerve fibers density (tyrosine hydroxylase (TH)-positive fibers). The anatomical distribution of markers of sympathetic innervation (TH) and inflammation (HLA-DR) significantly correlated with respective in vivo PET findings across left ventricle levels and regions. No changes were found in alpha-synuclein immunoreactivity. Additionally, CD36 protein expression was increased at the cardiomyocyte intercalated discs following PPARγ-activation compared to placebo and control groups. Systemic 6-OHDA decreased adrenal medulla expression of catecholamine producing enzymes (TH and aromatic L-amino acid decarboxylase) and circulating levels of norepinephrine, which were attenuated by PPARγ-activation. Overall, these results validate in vivo PET findings of cardiac sympathetic innervation, oxidative stress, and inflammation and illustrate cardiomyocyte CD36 upregulation as a marker of PPARγ target engagement.
心脏自主神经病变是帕金森病(PD)的一种常见非运动症状,与心脏交感神经支配丧失和血浆儿茶酚胺减少有关。目前尚无针对 PD 心脏神经退行性变的疾病修饰治疗策略,也缺乏靶向药物作用的生物标志物。全身给予儿茶酚胺神经毒素 6-羟多巴胺(6-OHDA)可重现 PD 心脏自主神经病变的病理过程。我们最近使用正电子发射断层扫描(PET)来可视化和量化成年雄性恒河猴(Macaca mulatta;n=10)接受 6-OHDA(50mg/kg;静脉注射)挑战后的心脏交感神经支配、氧化应激和炎症。在中毒后 24 小时,将动物盲目且随机分为每天接受过氧化物酶体增殖物激活受体γ(PPARγ)激动剂吡格列酮(n=5;5mg/kg 口服)或安慰剂(n=5)治疗。PET 放射性配体摄取的定量分析显示,6-OHDA 后一周氧化应激和炎症增加,12 周时恢复到基线水平,此时吡格列酮治疗的动物显示局部保留的交感神经支配。在这里,我们报告与年龄和性别匹配的正常对照(n=5)相比,这些动物的心脏和肾上腺组织的死后特征。在心脏中,6-OHDA 处理的动物显示出交感神经纤维密度(酪氨酸羟化酶(TH)阳性纤维)显著丧失。交感神经支配标志物(TH)和炎症(HLA-DR)的解剖分布与左心室水平和区域的相应体内 PET 发现显著相关。α-突触核蛋白免疫反应性无变化。此外,与安慰剂和对照组相比,PPARγ 激活后,在心肌闰盘处 CD36 蛋白表达增加。全身 6-OHDA 降低肾上腺髓质儿茶酚胺产生酶(TH 和芳香 L-氨基酸脱羧酶)的表达和循环去甲肾上腺素水平,PPARγ 激活可减轻这些变化。总的来说,这些结果验证了心脏交感神经支配、氧化应激和炎症的体内 PET 发现,并说明了心肌细胞 CD36 上调作为 PPARγ 靶标结合的标志物。