MTFR1L is a cardiac antiaging factor for maintenance of mitochondrial homeostasis

L Le Shi Z Zehui Sun (Shanghai Key Laboratory of Molecular Catalysis and Innovative Materials, Department of Chemistry) Y Yingxue Cao (State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University) Y Yuying Li W Wenjie Qing (State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University) L Lingli Zhou (Department of Ophthalmology, Johns Hopkins School of Medicine) M Mingming Xu (Division of Chemical Biology and Medicinal Chemistry, College of Pharmacy) X Xiaomei Mai (State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University) L Lechun Ou (State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University) X Xiaoping Yang (Anhui Province Key Laboratory of Low-Energy Quantum Materials and Devices, High Magnetic Field Laboratory, HFIPS) D Delaram Shakiba (NSF Science and Technology Center for Engineering Mechanobiology, Washington University in St. Louis) K Kaoru Ri (Department of Ophthalmology, Johns Hopkins School of Medicine) W Wangsheng Ou (State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University) J Jun Chen A Avi Rosenberg (Department of Pathology, Johns Hopkins Medical Institutions) J Jean-François Trempe E Edward A. Fon X Xialin Liu (State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University) Y Yihai Cao (Department of Microbiology, Tumor and Cell Biology, Karolinska Institutet) E Elia J. Duh (Department of Ophthalmology, Johns Hopkins School of Medicine) W Wei Yi (Obesity and Metabolism Medicine-Engineering Integration Laboratory, Department of General Surgery, The Affiliated Hospital of Southwest Jiaotong University, The Third People’s Hospital of Chengdu)

Abstract

Mitochondrial dysfunction is a hallmark of aging and a key contributor to age-related diseases including cardiovascular disease. However, molecular pathways that safeguard mitochondrial homeostasis in the aging heart remain poorly understood. Here, we identify MTFR1L as a regulator of mitophagy that binds p-S65-Ub, a key signal amplifying the PINK1/Parkin axis. We find that MTFR1L is enriched in metabolically active tissues, particularly in the heart, where it regulates Parkin signaling. Genetic deletion of Mtfr1l in mice impairs stress-induced mitophagy and Parkin activation, leading to accumulation of damaged mitochondria, increased inflammation and senescence, and accelerated age-related cardiac dysfunction. Strikingly, cardiac expression of MTFR1L progressively decreases along with aging in mice, primates, and humans, coinciding with cardiomyocyte senescence and lipofuscin accumulation. Together, these findings uncover a role for MTFR1L in regulation of the p-S65-Ub/Parkin mitophagy axis and maintenance of mitochondrial homeostasis during cardiac aging and suggest that age-associated loss of MTFR1L may contribute to age-related cardiac dysfunction. Based on these findings, we propose a therapeutic paradigm for the prevention of heart aging by restoring MTFR1L function.

Article Details

Volume / Issue Vol. 123, Issue 4
Published January 27, 2026
ISSN 0027-8424
Publisher National Academy of Sciences

Authors (21)

L

Le Shi

Z

Zehui Sun

Shanghai Key Laboratory of Molecular Catalysis and Innovative Materials, Department of Chemistry

Y

Yingxue Cao

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University

Y

Yuying Li

W

Wenjie Qing

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University

L

Lingli Zhou

Department of Ophthalmology, Johns Hopkins School of Medicine

M

Mingming Xu

Division of Chemical Biology and Medicinal Chemistry, College of Pharmacy

X

Xiaomei Mai

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University

L

Lechun Ou

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University

X

Xiaoping Yang

Anhui Province Key Laboratory of Low-Energy Quantum Materials and Devices, High Magnetic Field Laboratory, HFIPS

D

Delaram Shakiba

NSF Science and Technology Center for Engineering Mechanobiology, Washington University in St. Louis

K

Kaoru Ri

Department of Ophthalmology, Johns Hopkins School of Medicine

W

Wangsheng Ou

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University

J

Jun Chen

A

Avi Rosenberg

Department of Pathology, Johns Hopkins Medical Institutions

J

Jean-François Trempe

E

Edward A. Fon

X

Xialin Liu

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-Sen University

Y

Yihai Cao

Department of Microbiology, Tumor and Cell Biology, Karolinska Institutet

E

Elia J. Duh

Department of Ophthalmology, Johns Hopkins School of Medicine

W

Wei Yi

Obesity and Metabolism Medicine-Engineering Integration Laboratory, Department of General Surgery, The Affiliated Hospital of Southwest Jiaotong University, The Third People’s Hospital of Chengdu