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000282549 0247_ $$2ISSN$$a2473-9537
000282549 037__ $$aDZNE-2025-01312
000282549 041__ $$aEnglish
000282549 082__ $$a610
000282549 1001_ $$00000-0002-5353-7691$$aBruno, Mariolina$$b0
000282549 245__ $$aInterferon gamma rebalances immunopathological signatures in chronic granulomatous disease through metabolic rewiring.
000282549 260__ $$aWashington, DC$$bAmerican Society of Hematology$$c2025
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000282549 520__ $$aChronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent life-threatening infections and hyperinflammatory complications. It is caused by mutations in the reduced nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex and the consequent loss of reactive oxygen species (ROS) production. Recombinant human interferon gamma (rIFN-γ) prophylaxis reduces the risk of severe infections, but the mechanisms behind its efficacy in CGD are still an open question, as it does not restore NADPH oxidase-dependent ROS production. Here, we demonstrate that the innate immune cells of patients with CGD are transcriptionally and functionally reprogrammed to a hyperactive inflammatory status, displaying an impaired in vitro induction of trained immunity. CGD monocytes have reduced intracellular amino acid concentrations and profound functional metabolic defects, both at the level of glycolysis and mitochondrial respiration. Ex vivo and in vivo treatments with IFN-γ restored these metabolic defects and reduced excessive interleukin 1β (IL-1β) and IL-6 production in response to fungal stimuli in CGD monocytes. These data suggest that prophylactic rIFN-γ modulates the metabolic status of innate immune cells in CGD. These data shed light on the effects of NADPH oxidase-derived ROS deficiency to the metabolic programs of immune cells and pose the basis for targeting this immunometabolic axis, potentially beyond CGD, with IFN-γ immunotherapy.
000282549 536__ $$0G:(DE-HGF)POF4-354$$a354 - Disease Prevention and Healthy Aging (POF4-354)$$cPOF4-354$$fPOF IV$$x0
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000282549 588__ $$aDataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
000282549 650_7 $$082115-62-6$$2NLM Chemicals$$aInterferon-gamma
000282549 650_7 $$2NLM Chemicals$$aReactive Oxygen Species
000282549 650_7 $$0EC 1.6.3.-$$2NLM Chemicals$$aNADPH Oxidases
000282549 650_2 $$2MeSH$$aHumans
000282549 650_2 $$2MeSH$$aGranulomatous Disease, Chronic: metabolism
000282549 650_2 $$2MeSH$$aGranulomatous Disease, Chronic: immunology
000282549 650_2 $$2MeSH$$aGranulomatous Disease, Chronic: drug therapy
000282549 650_2 $$2MeSH$$aGranulomatous Disease, Chronic: pathology
000282549 650_2 $$2MeSH$$aInterferon-gamma: pharmacology
000282549 650_2 $$2MeSH$$aInterferon-gamma: therapeutic use
000282549 650_2 $$2MeSH$$aReactive Oxygen Species: metabolism
000282549 650_2 $$2MeSH$$aMonocytes: metabolism
000282549 650_2 $$2MeSH$$aMonocytes: immunology
000282549 650_2 $$2MeSH$$aMonocytes: drug effects
000282549 650_2 $$2MeSH$$aImmunity, Innate: drug effects
000282549 650_2 $$2MeSH$$aNADPH Oxidases: metabolism
000282549 650_2 $$2MeSH$$aMale
000282549 650_2 $$2MeSH$$aFemale
000282549 693__ $$0EXP:(DE-2719)PRECISE-20190321$$5EXP:(DE-2719)PRECISE-20190321$$ePlatform for Single Cell Genomics and Epigenomics at DZNE  University of Bonn$$x0
000282549 7001_ $$0P:(DE-2719)9000629$$aKröger, Charlotte$$b1
000282549 7001_ $$00000-0002-6274-0448$$aFerreira, Anaísa V$$b2
000282549 7001_ $$00000-0002-2911-9422$$aZhang, Bowen$$b3
000282549 7001_ $$00000-0003-3921-7541$$aRöring, Rutger J$$b4
000282549 7001_ $$00000-0001-9663-1357$$aLiu, Ruiqi$$b5
000282549 7001_ $$00000-0003-0763-4017$$avan der Made, Caspar I$$b6
000282549 7001_ $$00000-0001-6722-2757$$avan Rhijn, Norman$$b7
000282549 7001_ $$aGroh, Laszlo$$b8
000282549 7001_ $$aKlück, Viola$$b9
000282549 7001_ $$00000-0002-9629-8627$$aJanssen, Nico A F$$b10
000282549 7001_ $$aLi, Wenchao$$b11
000282549 7001_ $$aRosati, Diletta$$b12
000282549 7001_ $$00000-0003-1770-1332$$aAlaswad, Ahmed$$b13
000282549 7001_ $$00000-0002-5988-6147$$aTercan, Helin$$b14
000282549 7001_ $$aSaiz, Jorge$$b15
000282549 7001_ $$aGonzalez-Riano, Carolina$$b16
000282549 7001_ $$0P:(DE-2719)9002579$$avan Uelft, Martina$$b17$$udzne
000282549 7001_ $$00000-0002-4726-915X$$aGaal, Orsolya Ildiko$$b18
000282549 7001_ $$0P:(DE-2719)9001774$$aMüller, Sophie$$b19
000282549 7001_ $$0P:(DE-HGF)0$$aFerreira, Humberto J$$b20
000282549 7001_ $$0P:(DE-2719)9001511$$aWarnat-Herresthal, Stefanie$$b21$$udzne
000282549 7001_ $$0P:(DE-2719)2812750$$aBecker, Matthias$$b22
000282549 7001_ $$0P:(DE-2719)9001067$$aHolsten, Lisa$$b23$$udzne
000282549 7001_ $$0P:(DE-2719)9000840$$aKraut, Michael$$b24$$udzne
000282549 7001_ $$0P:(DE-2719)9001500$$aSchulte-Schrepping, Jonas$$b25$$udzne
000282549 7001_ $$0P:(DE-2719)9001512$$aBonaguro, Lorenzo$$b26
000282549 7001_ $$0P:(DE-2719)2812735$$aHändler, Kristian$$b27
000282549 7001_ $$aCunha, Cristina$$b28
000282549 7001_ $$aSchmolz, Manfred$$b29
000282549 7001_ $$0P:(DE-2719)2811660$$aSchultze, Joachim L$$b30
000282549 7001_ $$00000-0001-6166-9830$$aJoosten, Leo A B$$b31
000282549 7001_ $$00000-0003-4722-491X$$aBarbas, Coral$$b32
000282549 7001_ $$aNetea, Mihai G$$b33
000282549 7001_ $$00000-0003-4022-7341$$aLi, Yang$$b34
000282549 7001_ $$0P:(DE-2719)2812913$$aAschenbrenner, Anna C$$b35
000282549 7001_ $$00000-0001-8935-8030$$aCarvalho, Agostinho$$b36
000282549 7001_ $$avan de Veerdonk, Frank L$$b37
000282549 773__ $$0PERI:(DE-600)2876449-3$$a10.1182/bloodadvances.2025016213$$gVol. 9, no. 20, p. 5306 - 5322$$n20$$p5306 - 5322$$tBlood advances$$v9$$x2473-9529$$y2025
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