Cell type–specific purifying selection of synonymous mitochondrial DNA variation

C Caleb A. Lareau P Patrick Maschmeyer (Berlin Institute of Health at Charité—Universitätsmedizin Berlin) Y Yajie Yin (Department of Pathology, Stanford University) J Jacob C. Gutierrez R Ryan S. Dhindsa A Anne-Sophie Gribling-Burrer S Sebastian Zielinski (Helmholtz Institute for RNA-based Infection Research, Helmholtz-Center for Infection Research) Y Yu-Hsin Hsieh (Berlin Institute of Health at Charité—Universitätsmedizin Berlin) L Lena Nitsch V Veronika Dimitrova (Berlin Institute of Health at Charité—Universitätsmedizin Berlin) B Benan Nalbant (Computational and Systems Biology Program, Memorial Sloan Kettering Cancer Center) F Frank A. Buquicchio (Department of Pathology, Stanford University) T Tsion Abay (Department of Pathology, Stanford University) R Robert R. Stickels (Department of Pathology, Stanford University) J Jacob C. Ulirsch P Patrick Yan (Department of Pathology, Stanford University) F Fangyi Wang (Department of Pathology, Stanford University) Z Zhuang Miao K Katalin Sandor (Department of Pathology, Stanford University) B Bence Daniel (Department of Pathology, Stanford University) V Vincent Liu (Department of Pathology, Stanford University) P Paul L. Mendez (Department of Biology, Chemistry, Pharmacy, Freie Universität Berlin) P Petra Knaus (Department of Biology, Chemistry, Pharmacy, Freie Universität Berlin) M Manpreet Meyer (Berlin Institute of Health at Charité—Universitätsmedizin Berlin) W William J. Greenleaf A Anshul Kundaje R Redmond P. Smyth M Mathias Munschauer (Helmholtz Institute for RNA-based Infection Research, Helmholtz-Center for Infection Research) L Leif S. Ludwig A Ansuman T. Satpathy

Abstract

While somatic variants are well-characterized drivers of tumor evolution, their influence on cellular fitness in nonmalignant contexts remains understudied. We identified a mosaic synonymous variant (m.7076A > G) in the mitochondrial DNA (mtDNA)-encoded cytochrome c-oxidase subunit 1 (MT-CO1, p.Gly391=), present at homoplasmy in 47% of immune cells from a healthy donor. Single-cell multiomics revealed strong, lineage-specific selection against the m.7076G allele in CD8 + effector memory T cells, but not other T cell subsets, mirroring patterns of purifying selection of pathogenic mtDNA alleles. The limited anticodon diversity of mitochondrial tRNAs forces m.7076G translation to rely on wobble pairing, unlike the Watson–Crick–Franklin pairing used for m.7076A. Mitochondrial ribosome profiling confirmed stalled translation of the m.7076G allele. Functional analyses demonstrated that the elevated translational and metabolic demands of short-lived effector T cells (SLECs) amplify dependence on MT-CO1, driving this selective pressure. These findings suggest that synonymous variants can alter codon syntax, impacting mitochondrial physiology in a cell type–specific manner.

Article Details

Volume / Issue Vol. 122, Issue 30
Published July 29, 2025
ISSN 0027-8424
Publisher National Academy of Sciences

Authors (30)

C

Caleb A. Lareau

P

Patrick Maschmeyer

Berlin Institute of Health at Charité—Universitätsmedizin Berlin

Y

Yajie Yin

Department of Pathology, Stanford University

J

Jacob C. Gutierrez

R

Ryan S. Dhindsa

A

Anne-Sophie Gribling-Burrer

S

Sebastian Zielinski

Helmholtz Institute for RNA-based Infection Research, Helmholtz-Center for Infection Research

Y

Yu-Hsin Hsieh

Berlin Institute of Health at Charité—Universitätsmedizin Berlin

L

Lena Nitsch

V

Veronika Dimitrova

Berlin Institute of Health at Charité—Universitätsmedizin Berlin

B

Benan Nalbant

Computational and Systems Biology Program, Memorial Sloan Kettering Cancer Center

F

Frank A. Buquicchio

Department of Pathology, Stanford University

T

Tsion Abay

Department of Pathology, Stanford University

R

Robert R. Stickels

Department of Pathology, Stanford University

J

Jacob C. Ulirsch

P

Patrick Yan

Department of Pathology, Stanford University

F

Fangyi Wang

Department of Pathology, Stanford University

Z

Zhuang Miao

K

Katalin Sandor

Department of Pathology, Stanford University

B

Bence Daniel

Department of Pathology, Stanford University

V

Vincent Liu

Department of Pathology, Stanford University

P

Paul L. Mendez

Department of Biology, Chemistry, Pharmacy, Freie Universität Berlin

P

Petra Knaus

Department of Biology, Chemistry, Pharmacy, Freie Universität Berlin

M

Manpreet Meyer

Berlin Institute of Health at Charité—Universitätsmedizin Berlin

W

William J. Greenleaf

A

Anshul Kundaje

R

Redmond P. Smyth

M

Mathias Munschauer

Helmholtz Institute for RNA-based Infection Research, Helmholtz-Center for Infection Research

L

Leif S. Ludwig

A

Ansuman T. Satpathy