波形蛋白
成纤维细胞
衰老
细胞生物学
蛋白质稳态
中间丝蛋白
生物
蛋白质亚单位
癌症研究
伴侣(临床)
肺纤维化
纤维化
肺
中间灯丝
免疫学
化学
早衰
肺纤维化
细胞
分子生物学
作者
Ting Dong,Jiao Xue,Huirui Wang,Yinghui Gao,Yirong Xu,Hui Li,Senbiao Fang,Xinyi Chen,Mengmeng Wang,Hanbing Zhu,Nianyu Li,Bo Han,Mei Qi,Kaige Lyu,Kaicheng Ma,Ke Li,Haigen Fu,Bowen Gu,W Li,Yingying Qin
标识
DOI:10.1073/pnas.2426723123
摘要
Progressive lung fibrosis is linked to aging-related dysfunction in fibroblasts, which remains poorly understood. To investigate the alterations in fibroblasts, particularly the molecular programs driving this profibrotic evolution in the aging lung, we isolated senescent lung fibroblasts from aged mice. We observed aberrant vimentin aggregates, which correlate with accelerated fibroblast senescence. CRISPR-based screening identified calumenin as a chaperone protein essential for vimentin proteostasis. A fibroblast-specific knockout of calumenin promotes the accumulation of vimentin aggregates and profibrotic factors migracytosis, exacerbating fibroblast senescence and lung aging. Mechanistically, calumenin collaborates with the TRiC complex to facilitate proper vimentin folding and recruits the chaperonin subunit Chaperonin Containing TCP1 Subunit 2 (CCT2) to degrade misfolded vimentin aggregates. Pathologically, external profibrotic stimuli trigger calcium transients and induce calumenin degradation, resulting in fibroblast senescence and the initiation of fibrosis. The natural product 9-85, derived from high-content screening, specifically targets and disrupts vimentin aggregates upon stimulation, alleviating aging-related lung fibrosis. Our findings reveal that calumenin coordinates vimentin quality control to shape cell structure and suppress the secretome of senescent fibroblasts, providing a promising therapeutic strategy for aging-related organ fibrosis.
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