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Activated ATF6α is a hepatic tumour driver restricting immunosurveillance.

Nature 2026 Vol.651(8106) p. 796-807

Li X, Lebeaupin C, Kadianaki A, Druelle-Cedano C, Vesper N, Rennert C, Huguet-Pradell J, Gomez Ramos B, Fan C, Piecyk RS, Zizmare L, Ramadori P, Li L, Frick L, Qiu M, Zhang C, Martins Nascentes Melo L, Ranvir VP, Shen P, Hanselmann J, Kosla J, Fernández-Vaquero M, Vucur M, Baskaran P, Bao X, Coleman OI, Tang Y, Cetin M, Chen Z, Jang I, Del Prete S, Rahbari M, Zhang P, Pham TV, Hou Y, Sun A, Gu L, Kim LC, Rothermel U, Heide D, Ali A, Gallage S, Talvard-Balland N, Piqué-Gili M, Gris-Oliver A, Bevilacqua A, Schlicker L, Duffey A, Unger K, Szydlowska M, Hetzer J, Odom DT, Machauer T, Bucci D, Sant P, Lee JH, Rösler J, Meckelmann SW, Schreck J, Murray S, Simon MC, Nahnsen S, Schulze A, Ho PC, Jugold M, Breuhahn K, Mallm JP, Schirmacher P, Roth S, Rahbari N, Tschaharganeh DF, Roessler S, Goeppert B, Bengsch B, Andrieux G, Boerries M, Malek NP, Prinz M, Weber A, Zeiser R, Tamayo P, Bronsert P, Kurowski K, Thimme R, Yuan D, Carretero R, Luedde T, Pinyol R, Hartmann FJ, Karin M, Tasdogan A, Trautwein C, Mall M, Hofmann M, Llovet JM, Haller D, Kaufman RJ, Heikenwälder M

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Hepatocellular carcinoma (HCC) is the fastest growing cause of cancer-related mortality and there are limited therapies.

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APA Li X, Lebeaupin C, et al. (2026). Activated ATF6α is a hepatic tumour driver restricting immunosurveillance.. Nature, 651(8106), 796-807. https://doi.org/10.1038/s41586-025-10036-8
MLA Li X, et al.. "Activated ATF6α is a hepatic tumour driver restricting immunosurveillance.." Nature, vol. 651, no. 8106, 2026, pp. 796-807.
PMID 41639449

Abstract

Hepatocellular carcinoma (HCC) is the fastest growing cause of cancer-related mortality and there are limited therapies. Although endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) are implicated in HCC, the involvement of the UPR transducer ATF6α remains unclear. Here we demonstrate the function of ATF6α as an ER-stress-inducing tumour driver and metabolic master regulator restricting cancer immunosurveillance for HCC, in contrast to its well-characterized role as an adaptive response to ER stress. ATF6α activation in human HCC is significantly correlated with an aggressive tumour phenotype, characterized by reduced patient survival, enhanced tumour progression and local immunosuppression. Hepatocyte-specific ATF6α activation in mice induced progressive hepatitis with ER stress, immunosuppression and hepatocyte proliferation. Concomitantly, activated ATF6α increased glycolysis and directly repressed the gluconeogenic enzyme FBP1 by binding to gene regulatory elements. Restoring FBP1 expression limited ATF6α-activation-related pathologies. Prolonged ATF6α activation in hepatocytes triggered hepatocarcinogenesis, intratumoural T cell infiltration and nutrient-deprived immune exhaustion. Immune checkpoint blockade (ICB) restored immunosurveillance and reduced HCC. Consistently, patients with HCC who achieved a complete response to immunotherapy displayed significantly increased ATF6α activation compared with those with a weaker response. Targeting Atf6 through germline ablation, hepatocyte-specific ablation or therapeutic hepatocyte delivery of antisense oligonucleotides dampened HCC in preclinical liver cancer models. Thus, prolonged ATF6α activation drives ER stress, leading to glycolysis-dependent immunosuppression in liver cancer and sensitizing to ICB. Our findings suggest that persistently activated ATF6α is a tumour driver, a potential stratification marker for ICB response and a therapeutic target for HCC.

MeSH Terms

Liver Neoplasms; Animals; Activating Transcription Factor 6; Carcinoma, Hepatocellular; Mice; Humans; Hepatocytes; Endoplasmic Reticulum Stress; Female; Immunologic Surveillance; Male; Monitoring, Immunologic; Glycolysis; Immune Checkpoint Inhibitors; Unfolded Protein Response; Cell Proliferation; Carcinogenesis; Immunotherapy

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