A metabolism-specific drug-repurposing screen reveals itraconazole as a potent OXPHOS inhibitor in acute myeloid leukemia

E Ekaterini Himonas (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) L Lucie de Beauchamp (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) D Désirée Zerbst (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) E Eudoxie Desmares-Romain (3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France) D Daniele Sarnello (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) E Eric R. Kalkman (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) K Kevin M. Rattigan (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) D Daniel James E Engy Shokry M Mhairi Copland (7Paul O’Gorman Leukaemia Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom) E Emmanuel Griessinger (3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France) C Christian Récher V Véronique De Mas (3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France) F Francois Vergez (3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France) D David Sumpton A Aaron D. Schimmer M Mark D. Minden (Princess Margaret Cancer Centre, University Health Network) E Eyal Gottlieb E Emma Shanks (6Cancer Research UK Scotland Institute, Glasgow, United Kingdom) J Jean-Emmanuel Sarry G G. Vignir Helgason (1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom)

Abstract

Abstract Targeting mitochondrial oxidative phosphorylation (OXPHOS) enhances the effects of standard chemotherapy and overcomes treatment resistance in preclinical models of acute myeloid leukemia (AML). So far, the few clinically available OXPHOS inhibitors have shown adverse effects or limited potency in clinical trials; therefore, the identification of safe and effective drugs that target mitochondrial metabolism in AML is critical. Here, we performed a high-throughput drug-repurposing screen designed to identify clinically applicable OXPHOS-specific inhibitors through nutrient sensing. We uncover itraconazole, a US Food and Drug Administration–approved antifungal compound, as a potent OXPHOS inhibitor in AML cells. Mechanistically, through stable isotope-assisted metabolomics and functional studies, we reveal that cytochrome P450 family 51 subfamily A member 1 (CYP51A1), which is part of the cytochrome P450 family and the prime target of azole antifungals, is involved in mitochondrial respiration and electron transport chain (ETC) complex I activity in AML cells. Critically, we demonstrate that itraconazole and related azole antifungals interfere with tricarboxylic acid cycle activity and inhibit OXPHOS through the inhibition of ETC complex I activity. Overexpression of yeast nicotinamide adenine dinucleotide (NADH) dehydrogenase-1 (NDI1) restored mitochondrial NADH oxidation and complex I activity following itraconazole treatment. Using patient-derived cells and preclinical xenograft models, we demonstrate that itraconazole targets therapy-resistant leukemic stem cells (LSCs) when used in combination with cytarabine, highlighting the repurposing potential for itraconazole as a clinically safe and effective therapeutic option for AML LSC eradication.

Article Details

Journal Blood
Volume / Issue Vol. 147, Issue 21
Published May 21, 2026
Pages 2431-2447
ISSN 0006-4971
Publisher Elsevier BV

Journal Info

Blood

Elsevier BV

ISSN: 0006-4971 Health Sciences

Authors (21)

E

Ekaterini Himonas

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

L

Lucie de Beauchamp

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

D

Désirée Zerbst

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

E

Eudoxie Desmares-Romain

3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France

D

Daniele Sarnello

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

E

Eric R. Kalkman

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

K

Kevin M. Rattigan

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

D

Daniel James

E

Engy Shokry

M

Mhairi Copland

7Paul O’Gorman Leukaemia Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom

E

Emmanuel Griessinger

3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France

C

Christian Récher

V

Véronique De Mas

3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France

F

Francois Vergez

3Centre de Recherches en Cancérologie de Toulouse, Université de Toulouse, INSERM U1037, Centre National de la Recherche Scientifique U5077, Toulouse, France

D

David Sumpton

A

Aaron D. Schimmer

M

Mark D. Minden

Princess Margaret Cancer Centre, University Health Network

E

Eyal Gottlieb

E

Emma Shanks

6Cancer Research UK Scotland Institute, Glasgow, United Kingdom

J

Jean-Emmanuel Sarry

G

G. Vignir Helgason

1Wolfson Wohl Cancer Research Centre, School of Cancer Sciences, University of Glasgow, Glasgow, United Kingdom