Diagnostic and Research Institute of Pathology, Medical University of Graz, Graz, Austria.
BioTechMed-Graz, Graz, Austria.
JCI Insight. 2022 May 9;7(9):e149438. doi: 10.1172/jci.insight.149438.
The lung airways are constantly exposed to inhaled toxic substances, resulting in cellular damage that is repaired by local expansion of resident bronchiolar epithelial club cells. Disturbed bronchiolar epithelial damage repair lies at the core of many prevalent lung diseases, including chronic obstructive pulmonary disease, asthma, pulmonary fibrosis, and lung cancer. However, it is still not known how bronchiolar club cell energy metabolism contributes to this process. Here, we show that adipose triglyceride lipase (ATGL), the rate-limiting enzyme for intracellular lipolysis, is critical for normal club cell function in mice. Deletion of the gene encoding ATGL, Pnpla2 (also known as Atgl), induced substantial triglyceride accumulation, decreased mitochondrial numbers, and decreased mitochondrial respiration in club cells. This defect manifested as bronchiolar epithelial thickening and increased airway resistance under baseline conditions. After naphthalene‑induced epithelial denudation, a regenerative defect was apparent. Mechanistically, dysfunctional PPARα lipid-signaling underlies this phenotype because (a) ATGL was needed for PPARα lipid-signaling in regenerating bronchioles and (b) administration of the specific PPARα agonist WY14643 restored normal bronchiolar club cell ultrastructure and regenerative potential. Our data emphasize the importance of the cellular energy metabolism for lung epithelial regeneration and highlight the significance of ATGL-mediated lipid catabolism for lung health.
气道不断暴露于吸入的有毒物质,导致细胞损伤,而局部扩张的细支气管上皮板层细胞俱乐部细胞对损伤进行修复。细支气管上皮损伤修复障碍是许多常见肺部疾病的核心,包括慢性阻塞性肺疾病、哮喘、肺纤维化和肺癌。然而,目前尚不清楚细支气管板层细胞的能量代谢如何参与这一过程。在这里,我们发现脂肪甘油三酯脂肪酶(ATGL)是细胞内脂肪分解的限速酶,对小鼠的正常板层细胞功能至关重要。编码 ATGL 的基因 Pnpla2(也称为 Atgl)缺失导致大量甘油三酯积累、线粒体数量减少和线粒体呼吸减少。这一缺陷表现为在基础条件下细支气管上皮增厚和气道阻力增加。在萘诱导的上皮剥落后,再生缺陷明显。这种表型的机制是由于(a)ATGL 是再生细支气管中 PPARα 脂质信号所必需的,以及(b)特定的 PPARα 激动剂 WY14643 的给药恢复了正常的细支气管板层细胞超微结构和再生潜能。我们的数据强调了细胞能量代谢对于肺上皮再生的重要性,并强调了 ATGL 介导的脂肪分解代谢对于肺健康的意义。