Mitochondrial double-stranded RNA fuels pancreatic cancer growth via RIG-I/TLR3 inflammation

A Andrew T. Milcarek (Genome Regulation and Cell Signaling Program, The Wistar Institute) M Minjeong Yeon (Genome Regulation and Cell Signaling Program, The Wistar Institute) C Camilla Esposito (Genome Regulation and Cell Signaling Program, The Wistar Institute) P Prerna Kulkarni (Genome Regulation and Cell Signaling Program, The Wistar Institute) A Andrew V. Kossenkov J Jozef Madzo (Genome Regulation and Cell Signaling Program, The Wistar Institute) A Anneliese M. Faustino (Proteomics Shared Resource, The Wistar Institute) H Hsin-Yao Tang A Alessandra M. Storaci (Department of Pathophysiology and Transplantation, University of Milan) A Alessandro Palleschi (Division of Pathology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca’ Granda-Ospedale Maggiore) M Marco Locatelli (Division of Thoracic Surgery and Lung Transplantation, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca’ Granda-Ospedale Maggiore Policlinico) V Valentina Vaira (Department of Pathophysiology and Transplantation, University of Milan) M Mary V. Iacocca (Department of Pathology, Helen F. Graham Cancer Center and Research Institute, Christiana Care Health) A Andrea Ward (Department of Pathology, Helen F. Graham Cancer Center and Research Institute, Christiana Care Health) A Arvind Sabesan (Helen F. Graham Cancer Center and Research Institute, Christiana Care Health) N Nicholas J. Petrelli (Helen F. Graham Cancer Center and Research Institute, Christiana Care Health) M Michela Perego (Genome Regulation and Cell Signaling Program, The Wistar Institute) D Dario C. Altieri (Genome Regulation and Cell Signaling Program, The Wistar Institute)

Abstract

Mitochondria activate inflammation and innate immunity to protect against infections, but the role in cancer is unknown. Here, we report that patients with pancreatic ductal adenocarcinoma (PDAC) with reduced levels of the mitochondrial scaffold, Mic60, or inner mitochondrial membrane protein, exhibit increased inflammation, high NFκB activity and production of TNFα. This is mediated by double-stranded RNA (dsRNA) released from structurally defective, Mic60-low mitochondria, which engages TLR3/RIG-I sensing, activates NFκB gene expression and reprograms transcriptional and signaling networks to promote PDAC proliferation. Preclinical targeting of mitochondrial dsRNA signaling triggers rapid cell death and inhibition of tumor growth, selectively in Mic60-knockdown PDAC, without overt toxicity, in vivo. Therefore, dsRNA released from defective mitochondria generates protumorigenic inflammation and provides an actionable therapeutic target in selected PDAC patients.

Article Details

Volume / Issue Vol. 123, Issue 18
Published May 05, 2026
ISSN 0027-8424
Publisher National Academy of Sciences

Authors (18)

A

Andrew T. Milcarek

Genome Regulation and Cell Signaling Program, The Wistar Institute

M

Minjeong Yeon

Genome Regulation and Cell Signaling Program, The Wistar Institute

C

Camilla Esposito

Genome Regulation and Cell Signaling Program, The Wistar Institute

P

Prerna Kulkarni

Genome Regulation and Cell Signaling Program, The Wistar Institute

A

Andrew V. Kossenkov

J

Jozef Madzo

Genome Regulation and Cell Signaling Program, The Wistar Institute

A

Anneliese M. Faustino

Proteomics Shared Resource, The Wistar Institute

H

Hsin-Yao Tang

A

Alessandra M. Storaci

Department of Pathophysiology and Transplantation, University of Milan

A

Alessandro Palleschi

Division of Pathology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca’ Granda-Ospedale Maggiore

M

Marco Locatelli

Division of Thoracic Surgery and Lung Transplantation, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca’ Granda-Ospedale Maggiore Policlinico

V

Valentina Vaira

Department of Pathophysiology and Transplantation, University of Milan

M

Mary V. Iacocca

Department of Pathology, Helen F. Graham Cancer Center and Research Institute, Christiana Care Health

A

Andrea Ward

Department of Pathology, Helen F. Graham Cancer Center and Research Institute, Christiana Care Health

A

Arvind Sabesan

Helen F. Graham Cancer Center and Research Institute, Christiana Care Health

N

Nicholas J. Petrelli

Helen F. Graham Cancer Center and Research Institute, Christiana Care Health

M

Michela Perego

Genome Regulation and Cell Signaling Program, The Wistar Institute

D

Dario C. Altieri

Genome Regulation and Cell Signaling Program, The Wistar Institute