Article
Physiology
Sehwan Jang, Xavier R. Chapa-Dubocq, Silvia Fossati, Sabzali Javadov
Summary: The study compared the quantification of mitochondrial CRC in isolated cell mitochondria and permeabilized cells, demonstrating higher CRC in saponin-permeabilized cells. This indicates that quantifying mitochondrial CRC in cultured cells permeabilized by saponin has more advantages compared to isolated mitochondria.
FRONTIERS IN PHYSIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Shyamalagauri Jadhav, Olga Protchenko, Fengmin Li, Ethan Baratz, Minoo Shakoury-Elizeh, Alan Maschek, James Cox, Caroline C. Philpott
Summary: Iron is an essential nutrient that forms cofactors required for cellular protein activity, but can be toxic if not properly managed. PCBP1 is a multifunctional protein that binds iron and nucleic acids, regulating their fate. Deletion of PCBP1 in the liver results in dysregulated iron balance, leading to chronic liver disease with lipid accumulation and mitochondrial dysfunction. Supplementation with coenzyme Q is necessary to restore mitochondrial function in this context of ongoing oxidative damage.
FREE RADICAL BIOLOGY AND MEDICINE
(2021)
Article
Biochemistry & Molecular Biology
Ye Tian, Wei Lu, Ruicheng Shi, Reagan Mcguffee, Richard Lee, David A. Ford, Bo Wang
Summary: The study reveals that targeting LPCAT3 could be a novel therapy for insulin resistance, as suppression of Lpcat3 expression improves insulin sensitivity and metabolic issues in diabetic mice.
Article
Multidisciplinary Sciences
Gregory A. Wyant, Wenyu Yu, IIias P. Doulamis, Rio S. Nomoto, Mossab Y. Saeed, Thomas Duignan, James D. McCully, William G. Kaelin Jr
Summary: Kynurenic acid (KynA) has tissue-protective effects in ischemia models, and this study demonstrates that activation of GPR35 and interaction with ATPIF1 may be the underlying mechanism. These findings provide a rationale for developing specific GPR35 agonists for the treatment of ischemic diseases.
Article
Reproductive Biology
Alexis Ouellette, Mala Mahendroo, Shanmugasundaram Nallasamy
Summary: This study aimed to investigate the remodeling and hormonal regulation of collagen and elastin fibers in the myometrium during pregnancy. The results showed that the gene and protein expressions of collagen and elastin were induced early in pregnancy and were differentially regulated by progesterone and estrogen. Additionally, imaging analysis revealed structural reorganization of collagen and elastin fibers during pregnancy.
BIOLOGY OF REPRODUCTION
(2022)
Article
Biology
Montserrat Vega, David Castillo, Laura de Cubas, Yirong Wang, Ying Huang, Elena Hidalgo, Margarita Cabrera
Summary: In this study, a genome-wide screening of the deletion mutant collection of fission yeast was conducted, and four mutants associated with mitochondrial proteolysis that exhibit opposite effects on lifespan were identified. The analysis revealed a positive correlation between increased respiration rate and prolonged lifespan, and it was found that the longevity of the mutants depended on mitophagy induction.
Article
Pharmacology & Pharmacy
Li-Zhen Qiu, Wei Zhou, Lan-Xin Yue, Yi-Hao Wang, Fei-Ran Hao, Peng-Yan Li, Yue Gao
Summary: This study demonstrated that repeated treatment with low-doses of aconitine can remodel mitochondrial function through the AMPK-OPA1-ATP5A1 pathway, leading to enhanced myocardial contraction in Neonatal Rat Ventricular Myocytes. The upregulation of ATP synthase alpha-subunit (ATP5A1) was found to be crucial in improving mitochondrial function and cellular ATP content in cardiomyocytes. Additionally, inhibiting AMPK with compound C could reverse the effects of aconitine on mitochondrial function, suggesting a potential mechanism for the cardiotonic effect induced by medicinal plants containing aconitine.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Maria Soledad Alvarez, Estefania Nunez, Marina Fuertes-Agudo, Carme Cucarella, Maria Fernandez-Velasco, Lisardo Bosca, Jesus Vazquez, Rodrigue Rossignol, Paloma Martin-Sanz, Marta Casado
Summary: This study suggests a new association between COX-2 and mitochondria, which may contribute to increased expression of respiratory chain complex IV proteins, enhanced respiratory capacity, and increased ATP levels in the hearts of COX-2 transgenic mice, thus providing protective effects against ischemia-reperfusion injury.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Haruna Tani, Kaori Ishikawa, Hiroaki Tamashiro, Emi Ogasawara, Takehiro Yasukawa, Shigeru Matsuda, Akinori Shimizu, Dongchon Kang, Jun-Ichi Hayashi, Fan-Yan Wei, Kazuto Nakada
Summary: This study established a mouse model carrying a pathogenic mutation in the mitochondrial tRNA gene, associated with metabolic syndrome-like symptoms. The mutation led to decreased protein levels and reduced Complex I activity in mitochondria, providing insights into the molecular basis of mutation-mediated mitochondrial disorders.
NUCLEIC ACIDS RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
Takao Tsujioka, Daisuke Sasaki, Atsuhito Takeda, Hideyoshi Harashima, Yuma Yamada
Summary: The therapeutic potential of a myocardial mitochondria-targeting liposome encapsulating the cardioprotective compound resveratrol was evaluated in this study. The liposome was readily taken up by myocardial cells and localized to intracellular mitochondria. It significantly activated cellular mitochondrial function without causing cellular toxicity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Biology
Enrico Radaelli, Charles-Antoine Assenmacher, Jillian Verrelle, Esha Banerjee, Florence Manero, Salim Khiati, Anais Girona, Guillermo Lopez-Lluch, Placido Navas, Marco Spinazzi
Summary: Impaired spermatogenesis and male infertility are common manifestations of mitochondrial diseases. This study investigates the mechanism behind these conditions using a mouse model deficient in the mitochondrial protease PARL. The study reveals that PARL deficiency leads to early testicular atrophy and a complete arrest of spermatogenesis, independent of neurodegeneration. The researchers also found severe mitochondrial defects and a decrease in GPX4 expression in the arrested spermatocytes, leading to ferroptosis - a regulated cell death modality. These findings suggest a potential role of ferroptosis in the pathogenesis of mitochondrial diseases and male infertility.
Article
Nutrition & Dietetics
Alexia Gomez Rodriguez, Emanuela Talamonti, Alba Naudi, Anastasia V. Kalinovich, Anna M. Pauter, Gustavo Barja, Tore Bengtsson, Anders Jacobsson, Reinald Pamplona, Irina G. Shabalina
Summary: The production of endogenous DHA is crucial for the normal function of mouse liver mitochondria and may be relevant for human metabolism as well.
Article
Microbiology
Nicoletta Guaragnella, Gennaro Agrimi, Pasquale Scarcia, Clelia Suriano, Isabella Pisano, Antonella Bobba, Cristina Mazzoni, Luigi Palmieri, Sergio Giannattasio
Summary: The mitochondrial RTG-dependent retrograde signaling plays a role in various environmental stresses, with regulators identified in Saccharomyces cerevisiae. The activity of the transcriptional complex Rtg1/3 is modulated by Hog1 in response to osmotic stress, and RTG pathway contributes to osmoadaptation in an HOG1-dependent manner. Impairment of RTG signaling causes a decrease in mitochondrial respiratory capacity exclusively under osmostress, suggesting possible sequential interaction between HOG1 and the RTG pathway in stress signaling cascade.
Article
Clinical Neurology
Asami Tanimura, Weixing Shen, David Wokosin, D. James Surmeier
Summary: The specific remodeling of the CLN projection to the striatum in a late-stage PD model was investigated using transgenic mice and optogenetic and circuit mapping approaches. The results demonstrated a reduction in excitatory synaptic currents in both direct pathway spiny projection neurons (dSPNs) and indirect pathway spiny projection neurons (iSPNs), with a greater reduction observed in dSPNs. Moreover, the currents through alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptors in dSPNs were significantly reduced. These findings suggest that the CLN projection undergoes pathway-specific remodeling, which may contribute to the hypokinetic features of PD.
MOVEMENT DISORDERS
(2022)
Article
Medicine, Research & Experimental
Huiwen Liu, Li Wang, Hao Xu, Bin Tan, Qin Yi, Hongrong Deng, Yunxia Chen, Bolin He, Jie Tian, Jing Zhu
Summary: This study reveals the important role of PINK1 in regulating mitochondrial quality in human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs). Deletion of PINK1 leads to mitochondrial structural breakdown and dysfunction, resulting in decreased cardiomyocyte maturation and inhibition of mitochondrial ATP synthesis. Furthermore, the expression of cytoskeleton-related proteins and proteins related to cardiac pathology is also affected. These findings provide insights into the mechanism of PINK1 in cardiomyocyte development and guide efforts to promote the maturation of hiPSC-CMs.
JOURNAL OF TRANSLATIONAL MEDICINE
(2023)
Article
Multidisciplinary Sciences
Bernd Schoepf, Hansi Weissensteiner, Georg Schaefer, Federica Fazzini, Pornpimol Charoentong, Andreas Naschberger, Bernhard Rupp, Liane Fendt, Valesca Bukur, Irina Giese, Patrick Sorn, Ana Carolina Sant'Anna-Silva, Javier Iglesias-Gonzalez, Ugur Sahin, Florian Kronenberg, Erich Gnaiger, Helmut Klocker
NATURE COMMUNICATIONS
(2020)
Article
Physiology
Florian Hoppel, Elisa Calabria, Dominik H. Pesta, Wilhelm Kantner-Rumplmair, Erich Gnaiger, Martin Burtscher
Summary: This study evaluated the impact of ultramarathon running on platelet mitochondrial function, finding an increase in oxidative stress in platelet mitochondria and damage to muscle and kidneys. The study discovered a relationship between PLT mitochondrial function, individual fitness levels, extreme physical and mental stresses, warranting further research.
FRONTIERS IN PHYSIOLOGY
(2021)
Article
Oncology
Ana Carolina B. Sant'Anna-Silva, Juan A. Perez-Valencia, Marco Sciacovelli, Claude Lalou, Saharnaz Sarlak, Laura Tronci, Efterpi Nikitopoulou, Andras T. Meszaros, Christian Frezza, Rodrigue Rossignol, Erich Gnaiger, Helmut Klocker
Summary: The study found that succinate has an anaplerotic effect that enhances the metabolism and oncogenic properties of malignant prostate cells by replenishing the TCA cycle and promoting biosynthesis, amino acid, and carbon metabolism. Succinate also stimulates oxygen consumption and increases mitochondrial affinity in malignant prostate cells. These findings are crucial for understanding the metabolic adaptations of prostate tumor cells and potential therapeutic interventions.
Article
Cell Biology
Florian Hoppel, Luiz Felipe Garcia-Souza, Wilhelm Kantner-Rumplmair, Martin Burtscher, Erich Gnaiger, Dominik Pesta, Elisa Calabria
Summary: Human blood cells provide a minimally invasive strategy for studying mitochondrial function. By using high-resolution respirometry, researchers can reliably assess changes in mitochondrial function in human platelets. The method's reliability was demonstrated through experiments conducted in a laboratory and during field studies, showing consistent results across different conditions.
Article
Biology
Caroline-J. Kruse, David Stern, Ange Mouithys-Mickalad, Ariane Niesten, Tatiana Art, Helene Lemieux, Dominique-M. Votion
Summary: Equine atypical myopathy is a seasonal intoxication associated with ingestion of toxins from sycamore maple seeds and seedlings, leading to inhibited fatty acid oxidation and decreased mitochondrial respiration. The high mortality rate of about 74% is likely due to severe mitochondrial bioenergetic impairment induced by the toxins. Further studies are needed to fully understand the mechanism of toxicity and potential therapeutic candidates.
Article
Biochemistry & Molecular Biology
Mostafa A. Aboouf, Julia Armbruster, Markus Thiersch, Max Gassmann, Axel Goedecke, Erich Gnaiger, Glen Kristiansen, Anne Bicker, Thomas Hankeln, Hao Zhu, Thomas A. Gorr
Summary: The expression of myoglobin in brown adipose tissue impacts brown adipocyte activity by increasing mitochondrial density and maximal respiration capacity, influencing the composition of the free fatty acid pool, and shifting the lipid droplet equilibrium towards smaller droplets. This leads to up-regulated expression of thermogenesis markers in BAT, resulting in a leaner phenotype compared to mice without myoglobin expression. These findings suggest potential new strategies for treating metabolic disorders related to energy expenditure.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
(2021)
Article
Biochemistry & Molecular Biology
Christine Fischer, Chiara Volani, Timea Komlodi, Markus Seifert, Egon Demetz, Lara Valente de Souza, Kristina Auer, Verena Petzer, Laura von Raffay, Patrizia Moser, Erich Gnaiger, Guenter Weiss
Summary: This study investigates the effects of dietary and genetic iron overload on mitochondrial function, revealing that iron accumulation promotes ROS production and impairs mitochondrial respiratory capacity. Differences in response to iron overload were observed in wildtype and genetic hemochromatosis model mice, suggesting the potential for iron reduction therapy to improve mitochondrial function.
Article
Biochemistry & Molecular Biology
Andrea Vernerova, Luiz Felipe Garcia-Souza, Ondrej Soucek, Milan Kostal, Vit Rehacek, Lenka Kujovska Krcmova, Erich Gnaiger, Ondrej Sobotka
Summary: Human platelets have multiple functions, but their examination is limited by the lack of standardized methods. Different isolation methods yield platelets with varying mitochondrial metabolic profiles, which can be resolved through simple washing steps.
Article
Biochemistry & Molecular Biology
Gergely Pallag, Sara Nazarian, Dora Ravasz, David Bui, Timea Komlodi, Carolina Doerrier, Erich Gnaiger, Thomas N. Seyfried, Christos Chinopoulos
Summary: This study demonstrates that ProDH-directed proline catabolism can generate sufficient CIII and CIV proton pumping to support ATP production by the F1FO-ATPase even under CI inhibition.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Lucie Zdrazilova, Hana Hansikova, Erich Gnaiger
Summary: In this study, we compared the respiration differences between attached and suspended cells using multiple measurement platforms. The results showed no differences in ROUTINE and LEAK respiration between attached and suspended cells. The O2k platform had a higher electron transfer capacity than the XF24, which was possibly due to the limitations of uncoupler titrations in the XF24 platform leading to underestimation. Short-term suspension did not affect respiratory activity and coupling control.
Review
Biochemistry & Molecular Biology
Helene Lemieux, Pierre U. Blier
Summary: Temperature changes have significant impacts on the adaptations and physiological functions of animals. Understanding the mechanisms of these adaptations is crucial for assessing the survival capacity of ectothermic organisms in the face of global climate change. Mitochondrial oxidative phosphorylation plays a vital role in this process, but further research is needed to explore its complex interactions with other pathways for a comprehensive understanding of aerobic metabolism.
Article
Biochemistry & Molecular Biology
Christine Fischer, Lara Valente de Souza, Timea Komlodi, Luiz F. Garcia-Souza, Chiara Volani, Piotr Tymoszuk, Egon Demetz, Markus Seifert, Kristina Auer, Richard Hilbe, Natascha Brigo, Verena Petzer, Malte Asshoff, Erich Gnaiger, Gunter Weiss
Summary: Iron is essential for metabolism and its deficiency can lead to metabolic dysfunction and anemia. In a rat model of iron deficiency anemia (IDA), mitochondrial respiration was increased in peripheral blood mononuclear cells (PBMCs) but remained unaffected in the liver. This suggests that PBMC mitochondrial function cannot be a valid surrogate for liver respiration. Iron deficiency resulted in different metabolic reprogramming in blood cells and liver tissue.
Article
Oncology
Omar Torres-Quesada, Carolina Doerrier, Sophie Strich, Erich Gnaiger, Eduard Stefan
Summary: This study examines the impact of a newly formulated human plasma-like media (HPLM) on cell growth, mitochondrial bioenergetics, and drug efficacy. It reveals that cells grown in HPLM display altered mitochondrial profiles and that an FDA-approved cancer drug has context-dependent side effects in HPLM. These findings emphasize the importance of selecting a more physiological cell culture medium for analyzing bioenergetic profiles and assessing drug efficacies.
Article
Biochemistry & Molecular Biology
Nadine Spielmann, Christina Schenkl, Timea Komlodi, Patricia da Silva-Buttkus, Estelle Heyne, Jana Rohde, Oana Amarie, Birgit Rathkolb, Erich Gnaiger, Torsten Doenst, Helmut Fuchs, Valerie Gailus-Durner, Martin Hrabe de Angelis, Marten Szibor
Summary: Deletion of the Uqcrh gene causes abnormalities in cardiac morphology and contractility in mice, but does not increase the production of mitochondrial reactive oxygen species. This study provides important information for understanding rare mitochondrial disorders.
Article
Biochemistry & Molecular Biology
Gregor Oemer, Marie-Luise Edenhofer, Yvonne Wohlfarter, Katharina Lackner, Geraldine Leman, Jakob Koch, Luiza H. D. Cardoso, Herbert H. Lindner, Erich Gnaiger, Sandrine Dubrac, Johannes Zschocke, Markus A. Keller
Summary: Research shows that the functionality and structure of mitochondria are strongly influenced by the type of lipids in the growth medium, with lipid environment affecting the composition of mitochondrial cardiolipins and favoring the incorporation of essential linoleic acid. This raises further questions on the link between membrane composition and respiratory control.
JOURNAL OF LIPID RESEARCH
(2021)