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SMIM20 promotes complex IV biogenesis and Ca2+ signaling in mice heart

A. Boshnakovska, JR. Pronto, T. Gall, A. Aich, J. Prochazka, Z. Nichtova, R. Sedlacek, I. Sobitov, S. Ainatzi, C. Lenz, DM. Katschinski, H. Urlaub, N. Voigt, P. Rehling, LS. Kremer

. 2025 ; 44 (6) : 115723. [pub] 20250520

Language English Country United States

Document type Journal Article

Persistent link   https://www.medvik.cz/link/bmc25015270

Mitochondria are key to cellular energetics, metabolism, and signaling. Their dysfunction is linked to devastating diseases, including mitochondrial disorders, diabetes, neurodegenerative diseases, cardiac disorders, and cancer. Here, we present a knockout mouse model lacking the complex IV assembly factor SMIM20/MITRAC7. SMIM20-/- mice display cardiac pathology with reduced heart weight and cardiac output. Heart mitochondria present with reduced levels of complex IV associated with increased complex I activity, have altered fatty acid oxidation, and display elevated levels of ROS production. Interestingly, mutant mouse ventricular myocytes show unphysiological Ca2+ handling, which can be attributed to the increase in mitochondrial ROS production. Our study presents an example of a tissue-specific phenotype in the context of OXPHOS dysfunction. Moreover, our data suggest a link between complex IV dysfunction and Ca2+ handling at the endoplasmic reticulum through ROS signaling.

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$a Boshnakovska, Angela $u Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany; German Center for Child and Adolescent Health (DZKJ), 37075 Göttingen, Germany
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$a SMIM20 promotes complex IV biogenesis and Ca2+ signaling in mice heart / $c A. Boshnakovska, JR. Pronto, T. Gall, A. Aich, J. Prochazka, Z. Nichtova, R. Sedlacek, I. Sobitov, S. Ainatzi, C. Lenz, DM. Katschinski, H. Urlaub, N. Voigt, P. Rehling, LS. Kremer
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$a Mitochondria are key to cellular energetics, metabolism, and signaling. Their dysfunction is linked to devastating diseases, including mitochondrial disorders, diabetes, neurodegenerative diseases, cardiac disorders, and cancer. Here, we present a knockout mouse model lacking the complex IV assembly factor SMIM20/MITRAC7. SMIM20-/- mice display cardiac pathology with reduced heart weight and cardiac output. Heart mitochondria present with reduced levels of complex IV associated with increased complex I activity, have altered fatty acid oxidation, and display elevated levels of ROS production. Interestingly, mutant mouse ventricular myocytes show unphysiological Ca2+ handling, which can be attributed to the increase in mitochondrial ROS production. Our study presents an example of a tissue-specific phenotype in the context of OXPHOS dysfunction. Moreover, our data suggest a link between complex IV dysfunction and Ca2+ handling at the endoplasmic reticulum through ROS signaling.
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$a Pronto, Julius Ryan $u Institute of Pharmacology and Toxicology, University Medical Center Göttingen, Georg August University Göttingen, 37075 Göttingen, Germany; German Center for Cardiovascular Research (DZHK), Partner Site Göttingen, 37075 Göttingen, Germany
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$a Gall, Tanja $u Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany
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$a Aich, Abhishek $u Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany
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$a Prochazka, Jan $u Czech Centre for Phenogenomics, Institute of Molecular Genetics of the CAS, 142 20 Prague, Czech Republic
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$a Nichtova, Zuzana $u Czech Centre for Phenogenomics, Institute of Molecular Genetics of the CAS, 142 20 Prague, Czech Republic
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$a Sedlacek, Radislav $u Czech Centre for Phenogenomics, Institute of Molecular Genetics of the CAS, 142 20 Prague, Czech Republic
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$a Sobitov, Izzatullo $u Institute of Pharmacology and Toxicology, University Medical Center Göttingen, Georg August University Göttingen, 37075 Göttingen, Germany; German Center for Cardiovascular Research (DZHK), Partner Site Göttingen, 37075 Göttingen, Germany; Cluster of Excellence "Multiscale Bioimaging: From Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, 37075 Göttingen, Germany
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$a Ainatzi, Sofia $u Bioanalytical Mass Spectrometry Group, Max Planck Institute for Multidisciplinary Sciences, 37077 Göttingen, Germany
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$a Lenz, Christof $u Bioanalytical Mass Spectrometry Group, Max Planck Institute for Multidisciplinary Sciences, 37077 Göttingen, Germany; Department of Clinical Chemistry, University Medical Center Göttingen, 37075 Göttingen, Germany
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$a Katschinski, Dörthe M $u Department of Cardiovascular Physiology, University Medical Center Göttingen, 37073 Göttingen, Germany
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$a Urlaub, Henning $u Bioanalytical Mass Spectrometry Group, Max Planck Institute for Multidisciplinary Sciences, 37077 Göttingen, Germany; Department of Clinical Chemistry, University Medical Center Göttingen, 37075 Göttingen, Germany
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$a Voigt, Niels $u Institute of Pharmacology and Toxicology, University Medical Center Göttingen, Georg August University Göttingen, 37075 Göttingen, Germany; German Center for Cardiovascular Research (DZHK), Partner Site Göttingen, 37075 Göttingen, Germany; Cluster of Excellence "Multiscale Bioimaging: From Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, 37075 Göttingen, Germany
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$a Rehling, Peter $u Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany; German Center for Child and Adolescent Health (DZKJ), 37075 Göttingen, Germany; Cluster of Excellence "Multiscale Bioimaging: From Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, 37075 Göttingen, Germany; Max Planck Institute for Multidisciplinary Science, 37077 Göttingen, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology, Translational Neuroinflammation and Automated Microscopy, 37075 Göttingen, Germany. Electronic address: peter.rehling@medizin.uni-goettingen.de
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$a Kremer, Laura S $u Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany. Electronic address: laura.kremer@med.uni-goettingen.de
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