Juliana Heidler

RTN4IP1 is essential for the final stages of mitochondrial complex I assembly and coenzyme Q biosynthesis

Monika Oláhová, Rachel M. Guerra, Jack J. Collier, Juliana Heidler et autres

Summary A biochemical deficiency of mitochondrial complex I (CI) underlies ∼30% of cases of primary mitochondrial disease, yet the inventory of molecular machinery required for CI assembly remains incomplete. We previously characterised patients with isolated CI deficiency caused by segregating variants in …

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The dynamic exchange of subunits in Complex I is essential for its function in differentiated cells

Ilka Wittig, Paulina Castaneda Tamez, Alfredo Cabrera‐Orefice, Juliana Heidler et autres

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Preconditioning With Tetrahydrobiopterin Improves Outcome After Spinal Cord Ischaemia in a Small Animal Model

Juliana Heidler, Alexandra Gratl, Daniela Lobenwein, Sabine Wipper et autres

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Maintenance on mitochondrial complexes ensures bioenergetic function in differentiated cells

Ilka Wittig, Juliana Heidler, Heiko Giese, Ralf P. Brandes

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Genetic deletion of Nox4 enhances cancerogen-induced formation of solid tumors

Valeska Helfinger, Florian Freiherr von Gall, Nina Henke, Michael M. Kunze et autres

maintains basal activity of cellular surveillance systems and is unlikely to be cancerogenic. Utilizing two different murine models of cancerogen-induced solid tumors, we found that deletion of Nox4 promotes tumor formation and lowers recognition of DNA damage. Nox4 supports phosphorylation of H2AX …

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Impaired complex I repair causes recessive Leber’s hereditary optic neuropathy

Sarah L. Stenton, Н Л Шеремет, Claudia B. Catarino, N.A. Andreeva et autres

Leber's hereditary optic neuropathy (LHON) is the most frequent mitochondrial disease and was the first to be genetically defined by a point mutation in mitochondrial DNA (mtDNA). A molecular diagnosis is achieved in up to 95% of cases, the vast majority of …

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Mapping the Endothelial Cell S -Sulfhydrome Highlights the Crucial Role of Integrin Sulfhydration in Vascular Function

Sofia‐Iris Bibli, Jiong Hu, Mario Looso, Andreas Weigert et autres

Background: In vascular endothelial cells, cysteine metabolism by the cystathionine γ lyase (CSE), generates hydrogen sulfide–related sulfane sulfur compounds (H 2 S n ), that exert their biological actions via cysteine S -sulfhydration of target proteins. This study set out to map …

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Platelet-derived calpain cleaves the endothelial protease-activated receptor 1 to induce vascular inflammation in diabetes

Anastasia Kyselova, Amro Elgheznawy, Ilka Wittig, Juliana Heidler et autres

Abstract Diabetes mellitus is a major risk factor for cardiovascular disease. Platelets from diabetic patients are hyperreactive and release microparticles that carry activated cysteine proteases or calpains. Whether platelet-derived calpains contribute to the development of vascular complications in diabetes is unknown. Here …

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Mitochondrial Complexome Profiling

Heiko Giese, Jana Meisterknecht, Juliana Heidler, Ilka Wittig

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Bi‐allelic pathogenic variants in NDUFC2 cause early‐onset Leigh syndrome and stalled biogenesis of complex I

Ahmad Alahmad, Alessia Nasca, Juliana Heidler, Kyle Thompson et autres

Leigh syndrome is a progressive neurodegenerative disorder, most commonly observed in paediatric mitochondrial disease, and is often associated with pathogenic variants in complex I structural subunits or assembly factors resulting in isolated respiratory chain complex I deficiency. Clinical heterogeneity has been reported, …

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Redox Regulation of PPARγ in Polarized Macrophages

Verena Trümper, Ilka Wittig, Juliana Heidler, Florian Richter et autres

The peroxisome proliferator-activated receptor (PPARγ) is a central mediator of cellular lipid metabolism and immune cell responses during inflammation. This is facilitated by its role as a transcription factor as well as a DNA-independent protein interaction partner. We addressed how the cellular …

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Loss of mitochondrial ClpP, Lonp1, and Tfam triggers transcriptional induction of Rnf213, a susceptibility factor for moyamoya disease

Jana Key, Antonia Maletzko, Aneesha Kohli, Suzana Gispert et autres

Human RNF213, which encodes the protein mysterin, is a known susceptibility gene for moyamoya disease (MMD), a cerebrovascular condition with occlusive lesions and compensatory angiogenesis. Mysterin mutations, together with exposure to environmental trigger factors, lead to an elevated stroke risk since childhood. …

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