丙氨酸
肌成纤维细胞
谷氨酰胺
生物
谷氨酰胺分解
生物化学
特发性肺纤维化
细胞内
糖酵解
柠檬酸循环
纤维化
细胞生物学
化学
氨基酸
内科学
肺
新陈代谢
医学
作者
Fei Li,Niv Vigder,David R. Ziehr,Mari Kamiya,Hung N. Nguyen,Diana E. Ferreyra Faustino,Aseel H. Khalil,Hilaire C. Lam,Matthew L. Steinhauser,Edy Yong Kim,William M. Oldham
出处
期刊:JCI insight
[American Society for Clinical Investigation]
日期:2026-04-23
卷期号:11 (13)
被引量:4
标识
DOI:10.1172/jci.insight.199449
摘要
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease driven by aberrant fibroblast-to-myofibroblast differentiation, which requires metabolic reprogramming. Here, we identify alanine as an essential metabolite for myofibroblast differentiation. TGF-β increases intracellular alanine levels through enhanced synthesis and import in both normal and IPF lung fibroblasts. Alanine synthesis is primarily mediated by glutamate-pyruvate transaminase 2 (GPT2), whose expression is regulated by the glutamine/glutamate/α-ketoglutarate axis. Inhibition of GPT2 depletes alanine and suppresses TGF-β-induced α-SMA and COL1A1 expression, which are rescued by exogenous alanine. We also identify solute carrier family 38 member 2 (SLC38A2) as a transporter for both alanine and glutamine, upregulated by TGF-β or alanine deprivation. SLC38A2 and GPT2 form a coordinated regulatory axis sustaining intracellular alanine levels to support myofibroblast differentiation. Mechanistically, alanine deficiency impairs glycolytic flux and depletes tricarboxylic acid cycle intermediates, while alanine supplementation provides carbon and nitrogen for intracellular glutamate and proline biosynthesis, particularly under glutamine deprivation. Combined inhibition of alanine synthesis and uptake suppresses fibrogenic responses in fibroblasts and human precision-cut lung slices, highlighting dual metabolic targeting as a potential therapeutic strategy for fibrotic lung disease.
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