Glutaredoxin 2 is essential for AML survival through mitochondrial permeability transition pore regulation

T Tianyi Ling (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) C Cristiana O’Brien (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) J Jonathan R. St-Germain (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) V Vincent Rondeau (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) M Mary Shi (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) J Jacob M. Berman A Adrianna Cepa (3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH) P Paula Saez Raez (3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH) M Mark Wunderlich K Katharine M. Carter (3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH) C Cody Stillwell (3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH) C Christina Sexton (3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH) R Rachel Culp-Hill (7Department of Biochemistry and Molecular Genetics, University of Colorado Anschutz Medical Campus, Aurora, CO) J Julie A. Reisz S Saeer A. Adeel (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) A Andy G. X. Zeng S Suraj Bansal (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) E Emily Tsao (University of Toronto, Toronto, Ontario, Canada) H He Tian Chen (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) J John E. Dick M Mark D. Minden (Princess Margaret Cancer Centre, University Health Network) A Andrea Arruda (Princess Margaret Cancer Centre, University Health Network) M Maria L. Amaya (9Division of Hematology, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO) A Anastasia N. Tikhonova (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) K Kristin J. Hope (1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada) A Angelo D’Alessandro B Brian Raught C Courtney L. Jones (12Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH)

Abstract

Abstract Patients with acute myeloid leukemia (AML) have a poor 5-year survival rate, highlighting the need for the identification of new approaches to target this disease. AML is highly dependent on glutathione (GSH) metabolism for survival. Although the metabolic role of GSH is well characterized in AML, the contribution of protein glutathionylation, a reversible modification that protects protein thiols from oxidative damage, remains largely unexplored. Therefore, we sought to elucidate the role of protein glutathionylation in AML pathogenesis. Here, we demonstrate that protein glutathionylation is essential for AML cell survival. Specifically, the loss of glutaredoxin 2 (GLRX2), an enzyme that removes GSH modifications, resulted in selective primary AML cell death while sparing normal human hematopoietic stem and progenitor cells. Unbiased proteomic analysis revealed increased mitochondrial protein glutathionylation upon GLRX2 depletion, accompanied by mitochondrial dysfunction, including impaired oxidative phosphorylation, reduced mitochondrial membrane potential, and increased opening of the mitochondrial permeability transition pore (mPTP). Further investigation identified adenosine triphosphate synthase subunit O (ATP5PO), a key regulator of mPTP opening and a component of the ATP synthase complex, as a critical GLRX2 target. Disruption of ATP5PO glutathionylation partially restored mPTP function and rescued AML cell viability after GLRX2 depletion. Moreover, both genetic and pharmacological inhibition of mPTP opening restored the leukemic potential of primary AML specimens in the absence of GLRX2. By disrupting glutathionylation-dependent mitochondrial homeostasis, this study reveals a novel vulnerability in AML that could inform future therapeutic strategies.

Article Details

Journal Blood
Volume / Issue Vol. 147, Issue 8
Published February 19, 2026
Pages 863-876
ISSN 0006-4971
Publisher Elsevier BV

Journal Info

Blood

Elsevier BV

ISSN: 0006-4971 Health Sciences

Authors (28)

T

Tianyi Ling

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

C

Cristiana O’Brien

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

J

Jonathan R. St-Germain

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

V

Vincent Rondeau

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

M

Mary Shi

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

J

Jacob M. Berman

A

Adrianna Cepa

3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

P

Paula Saez Raez

3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

M

Mark Wunderlich

K

Katharine M. Carter

3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

C

Cody Stillwell

3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

C

Christina Sexton

3Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

R

Rachel Culp-Hill

7Department of Biochemistry and Molecular Genetics, University of Colorado Anschutz Medical Campus, Aurora, CO

J

Julie A. Reisz

S

Saeer A. Adeel

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

A

Andy G. X. Zeng

S

Suraj Bansal

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

E

Emily Tsao

University of Toronto, Toronto, Ontario, Canada

H

He Tian Chen

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

J

John E. Dick

M

Mark D. Minden

Princess Margaret Cancer Centre, University Health Network

A

Andrea Arruda

Princess Margaret Cancer Centre, University Health Network

M

Maria L. Amaya

9Division of Hematology, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO

A

Anastasia N. Tikhonova

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

K

Kristin J. Hope

1Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

A

Angelo D’Alessandro

B

Brian Raught

C

Courtney L. Jones

12Division of Experimental Hematology and Cancer Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH