Gookin Jody L, Mathews Kyle G, Cullen John, Seiler Gabriela
Department of Clinical Sciences, College of Veterinary Medicine and Comparative Medicine Institute, North Carolina State University, Raleigh, North Carolina, United States of America.
Department of Population Health and Pathobiology, College of Veterinary Medicine and Comparative Medicine Institute, North Carolina State University, Raleigh, North Carolina, United States of America.
PLoS One. 2018 Jan 11;13(1):e0191076. doi: 10.1371/journal.pone.0191076. eCollection 2018.
Mucocele formation is characterized by secretion of abnormally thick mucus by the gallbladder epithelium of dogs that may cause obstruction of the bile duct or rupture of the gallbladder. The disease is increasingly recognized and is associated with a high morbidity and mortality. The cause of gallbladder mucocele formation in dogs is unknown. There is a strong breed predisposition and affected dogs have a high incidence of concurrent endocrinopathy or hyperlipidemia. These observations suggest a significant influence of both genetic and metabolic factors on disease pathogenesis. In this study, we investigated a theory that mucocele formation is associated with a syndrome of metabolic disruption. We surmised that a global, untargeted metabolomics approach could provide unique insight into the systemic pathogenesis of gallbladder mucocele formation and identify specific compounds as candidate biomarkers or treatment targets. Moreover, concurrent examination of the serum and hepatic duct bile metabolome would enable the construction of mechanism-based theories or identification of specific compounds responsible for altered function of the gallbladder epithelium. Abnormalities observed in dogs with gallbladder mucocele formation, including a 33-fold decrease in serum adenosine 5'-monophosphate (AMP), lower quantities of precursors required for synthesis of energy transporting nucleotides, and increases in citric acid cycle intermediates, suggest excess metabolic energy and a carbon surplus. Altered quantities of compounds involved in protein translation and RNA turnover, together with accumulation of gamma-glutamylated and N-acetylated amino acids in serum suggest abnormal regulation of protein and amino acid metabolism. Increases in lathosterol and 7α-hydroxycholesterol suggest a primary increase in cholesterol synthesis and diversion to bile acid formation. A number of specific biomarker compounds were identified for their ability to distinguish between control dogs and those that formed a gallbladder mucocele. Particularly noteworthy was a significant decrease in quantity of biologically active compounds that stimulate biliary ductal fluid secretion including adenosine, cAMP, taurolithocholic acid, and taurocholic acid. These findings support the presence of significant metabolic disruption in dogs with mucocele formation. A targeted, quantitative analysis of the identified serum biomarkers is warranted to determine their utility for diagnosis of this disease. Finally, repletion of compounds whose biological activity normally promotes biliary ductal secretion should be examined for any therapeutic impact for resolution or prevention of mucocele formation.
黏液囊肿形成的特征是犬胆囊上皮分泌异常浓稠的黏液,这可能导致胆管阻塞或胆囊破裂。这种疾病越来越受到重视,且发病率和死亡率都很高。犬胆囊黏液囊肿形成的原因尚不清楚。该病存在强烈的品种易感性,患病犬同时患有内分泌病或高脂血症的发生率很高。这些观察结果表明遗传和代谢因素对疾病发病机制有重大影响。在本研究中,我们调查了一种理论,即黏液囊肿形成与代谢紊乱综合征有关。我们推测,一种全面的、非靶向的代谢组学方法可以为胆囊黏液囊肿形成的系统发病机制提供独特的见解,并识别出特定化合物作为候选生物标志物或治疗靶点。此外,同时检测血清和肝管胆汁代谢组将有助于构建基于机制的理论,或识别导致胆囊上皮功能改变的特定化合物。在患有胆囊黏液囊肿形成的犬中观察到的异常情况,包括血清5'-单磷酸腺苷(AMP)降低33倍、能量转运核苷酸合成所需前体数量减少以及柠檬酸循环中间体增加,提示代谢能量过剩和碳过剩。参与蛋白质翻译和RNA周转的化合物数量改变,以及血清中γ-谷氨酰化和N-乙酰化氨基酸的积累,提示蛋白质和氨基酸代谢的异常调节。羊毛甾醇和7α-羟基胆固醇增加提示胆固醇合成原发性增加并转向胆汁酸形成。鉴定出了一些特定的生物标志物化合物,它们能够区分对照犬和形成胆囊黏液囊肿的犬。特别值得注意的是,刺激胆管液分泌的生物活性化合物数量显著减少,包括腺苷、cAMP、牛磺石胆酸和牛磺胆酸。这些发现支持了黏液囊肿形成的犬存在显著代谢紊乱的观点。有必要对已鉴定的血清生物标志物进行靶向定量分析,以确定它们在诊断这种疾病中的效用。最后,应检查补充其生物活性通常促进胆管分泌的化合物对黏液囊肿形成的消退或预防是否有任何治疗作用。