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Mitochondrial supercomplexes

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https://www.readbyqxmd.com/read/28804536/combined-respiratory-chain-deficiency-and-uqcc2-mutations-in-neonatal-encephalomyopathy-defective-supercomplex-assembly-in-complex-iii-deficiencies
#1
René G Feichtinger, Michaela Brunner-Krainz, Bader Alhaddad, Saskia B Wortmann, Reka Kovacs-Nagy, Tatjana Stojakovic, Wolfgang Erwa, Bernhard Resch, Werner Windischhofer, Sarah Verheyen, Sabine Uhrig, Christian Windpassinger, Felix Locker, Christine Makowski, Tim M Strom, Thomas Meitinger, Holger Prokisch, Wolfgang Sperl, Tobias B Haack, Johannes A Mayr
Vertebrate respiratory chain complex III consists of eleven subunits. Mutations in five subunits either mitochondrial (MT-CYB) or nuclear (CYC1, UQCRC2, UQCRB, and UQCRQ) encoded have been reported. Defects in five further factors for assembly (TTC19, UQCC2, and UQCC3) or iron-sulphur cluster loading (BCS1L and LYRM7) cause complex III deficiency. Here, we report a second patient with UQCC2 deficiency. This girl was born prematurely; pregnancy was complicated by intrauterine growth retardation and oligohydramnios...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28794267/coi1-is-a-novel-assembly-factor-of-the-yeast-complex-iii-complex-iv-supercomplex
#2
Ravi K Singhal, Christine Kruse, Juliana Heidler, Valentina Strecker, Klaus Zwicker, Lea Düsterwald, Benedikt Westermann, Johannes M Herrmann, Ilka Wittig, Doron Rapaport
The yeast bc1 complex (complex III) and cytochrome oxidase (complex IV) are mosaics of core subunits encoded by the mitochondrial genome and additional nuclear-encoded proteins imported from the cytosol. Both complexes build in the mitochondrial inner membrane various supramolecular assemblies. The formation of the individual complexes and their supercomplexes depends on the activity of dedicated assembly factors. We identified a so far uncharacterized mitochondrial protein (open reading frame YDR381C-A) as an important assembly factor for complex III, complex IV, and their supercomplexes...
August 9, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28790391/deficiency-of-cox7rp-a-mitochondrial-supercomplex-assembly-promoting-factor-lowers-blood-glucose-level-in-mice
#3
Sachiko Shiba, Kazuhiro Ikeda, Kuniko Horie-Inoue, Akitoshi Nakayama, Tomoaki Tanaka, Satoshi Inoue
Mitochondria are essential organelles to efficiently produce ATP by ATP-synthase, which uses a proton-gradient generated by respiratory chain complexes. We previously demonstrated that COX7RP/COX7A2L/SCAF1 is a key molecule that promotes respiratory supercomplex assembly and regulates energy generation. The contribution of COX7RP to metabolic homeostasis, however, remains to be clarified. In the present study, we showed a metabolic phenotype of Cox7rp knockout (Cox7rpKO) mice, which exhibit lower blood glucose levels after insulin or pyruvate injection...
August 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28781720/mitochondrial-respiration-in-human-colorectal-and-breast-cancer-clinical-material-is-regulated-differently
#4
Andre Koit, Igor Shevchuk, Lyudmila Ounpuu, Aleksandr Klepinin, Vladimir Chekulayev, Natalja Timohhina, Kersti Tepp, Marju Puurand, Laura Truu, Karoliina Heck, Vahur Valvere, Rita Guzun, Tuuli Kaambre
We conducted quantitative cellular respiration analysis on samples taken from human breast cancer (HBC) and human colorectal cancer (HCC) patients. Respiratory capacity is not lost as a result of tumor formation and even though, functionally, complex I in HCC was found to be suppressed, it was not evident on the protein level. Additionally, metabolic control analysis was used to quantify the role of components of mitochondrial interactosome. The main rate-controlling steps in HBC are complex IV and adenine nucleotide transporter, but in HCC, complexes I and III...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28774815/antioxidant-defense-in-quiescent-cells-determines-selectivity-of-electron-transport-chain-inhibition-induced-cell-death
#5
Jan Blecha, Silvia Magalhaes Novais, Katerina Rohlenova, Eliska Novotna, Sandra Lettlova, Sabine Schmitt, Hans Zischka, Jiri Neuzil, Jakub Rohlena
Mitochondrial electron transport chain (ETC) targeting shows a great promise in cancer therapy. It is particularly effective in tumors with high ETC activity where ETC-derived reactive oxygen species (ROS) are efficiently induced. Why modern ETC-targeted compounds are tolerated on the organismal level remains unclear. As most somatic cells are in non-proliferative state, the features associated with the ETC in quiescence could account for some of the specificity observed. Here we report that quiescent cells, despite increased utilization of the ETC and enhanced supercomplex assembly, are less susceptible to cell death induced by ETC disruption when glucose is not limiting...
July 31, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28765075/parkinson-s-disease-associated-pathogenic-vps35-mutation-causes-complex-i-deficits
#6
Leping Zhou, Wenzhang Wang, Charles Hoppel, Jun Liu, Xiongwei Zhu
Defect in the complex I of the mitochondrial electron-transport chain is a characteristic of Parkinson's disease (PD) which is thought to play a critical role in the disease pathogenesis. Mutations in vacuolar sorting protein 35 (VPS35) cause autosomal dominant PD and we recently demonstrated that pathogenic VPS35 mutations cause mitochondrial damage through enhanced mitochondrial fragmentation. In this study, we aimed to determine whether pathogenic VPS35 mutation impacts the activity of complex I and its underlying mechanism...
July 29, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28747716/burn-induced-muscle-metabolic-derangements-and-mitochondrial-dysfunction-are-associated-with-activation-of-hif-1%C3%AE-and-mtorc1-role-of-protein-farnesylation
#7
Harumasa Nakazawa, Kazuhiro Ikeda, Shohei Shinozaki, Masayuki Kobayashi, Yuichi Ikegami, Ming Fu, Tomoyuki Nakamura, Shingo Yasuhara, Yong-Ming Yu, J A Jeevendra Martyn, Ronald G Tompkins, Kentaro Shimokado, Tomoko Yorozu, Hideki Ito, Satoshi Inoue, Masao Kaneki
Metabolic derangements are a clinically significant complication of major trauma (e.g., burn injury) and include various aspects of metabolism, such as insulin resistance, muscle wasting, mitochondrial dysfunction and hyperlactatemia. Nonetheless, the molecular pathogenesis and the relation between these diverse metabolic alterations are poorly understood. We have previously shown that burn increases farnesyltransferase (FTase) expression and protein farnesylation and that FTase inhibitor (FTI) prevents burn-induced hyperlactatemia, insulin resistance, and increased proteolysis in mouse skeletal muscle...
July 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28743641/respiratory-chain-supercomplexes-structures-function-and-biogenesis
#8
REVIEW
Teresa Lobo-Jarne, Cristina Ugalde
Over the past sixty years, researchers have made outmost efforts to clarify the structural organization and functional regulation of the complexes that configure the mitochondrial respiratory chain. As a result, the entire composition of each individual complex is practically known and, aided by notable structural advances in mammals, it is now widely accepted that these complexes stablish interactions to form higher-order supramolecular structures called supercomplexes and respirasomes. The mechanistic models and players that regulate the function and biogenesis of such superstructures are still under intense debate, and represent one of the hottest topics of the mitochondrial research field at present...
July 22, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28736227/association-between-ros-production-swelling-and-the-respirasome-integrity-in-cardiac-mitochondria
#9
Sehwan Jang, Sabzali Javadov
Although mitochondrial Ca(2+) overload and ROS production play a critical role in mitochondria-mediated cell death, a cause-effect relationship between them remains elusive. This study elucidated the crosstalk between mitochondrial swelling, ROS production, and electron transfer chain (ETC) supercomplexes in rat heart mitochondria in response to Ca(2+) and tert-butyl hydroperoxide (TBH), a lipid-soluble organic peroxide. Results showed that ROS production induced by TBH was significantly increased in the presence of Ca(2+) in a dose-dependent manner...
July 20, 2017: Archives of Biochemistry and Biophysics
https://www.readbyqxmd.com/read/28719865/antioxidant-effect-of-exercise-exploring-the-role-of-the-mitochondrial-complex-i-superassembly
#10
J R Huertas, S Al Fazazi, A Hidalgo-Gutierrez, L C López, R A Casuso
Mitochondrial respiratory complexes become assembled into supercomplexes (SC) under physiological conditions. One of the functional roles of these entities is the limitation of reactive oxygen species (ROS) produced by complex I (CI) of the respiratory chain. We sought to determine whether the systemic antioxidant effect of exercise is mediated by the assembly of mitochondrial CIs into SCs in rats. Male Wistar rats were exercise trained or remained sedentary for ten weeks; then, blood samples were collected, and the gastrocnemius muscle was isolated...
July 11, 2017: Redox Biology
https://www.readbyqxmd.com/read/28658629/definition-of-a-high-confidence-mitochondrial-proteome-at-quantitative-scale
#11
Marcel Morgenstern, Sebastian B Stiller, Philipp Lübbert, Christian D Peikert, Stefan Dannenmaier, Friedel Drepper, Uri Weill, Philipp Höß, Reinhild Feuerstein, Michael Gebert, Maria Bohnert, Martin van der Laan, Maya Schuldiner, Conny Schütze, Silke Oeljeklaus, Nikolaus Pfanner, Nils Wiedemann, Bettina Warscheid
Mitochondria perform central functions in cellular bioenergetics, metabolism, and signaling, and their dysfunction has been linked to numerous diseases. The available studies cover only part of the mitochondrial proteome, and a separation of core mitochondrial proteins from associated fractions has not been achieved. We developed an integrative experimental approach to define the proteome of yeast mitochondria. We classified > 3,300 proteins of mitochondria and mitochondria-associated fractions and defined 901 high-confidence mitochondrial proteins, expanding the set of mitochondrial proteins by 82...
June 27, 2017: Cell Reports
https://www.readbyqxmd.com/read/28654702/stomatin-like-protein-2-deficiency-results-in-impaired-mitochondrial-translation
#12
Panagiotis Mitsopoulos, Orsolya Lapohos, Woranontee Weraarpachai, Hana Antonicka, Yu-Han Chang, Joaquín Madrenas
Mitochondria translate the RNAs for 13 core polypeptides of respiratory chain and ATP synthase complexes that are essential for the assembly and function of these complexes. This process occurs in close proximity to the mitochondrial inner membrane. However, the mechanisms and molecular machinery involved in mitochondrial translation are not fully understood, and defects in this process can result in severe diseases. Stomatin-like protein (SLP)-2 is a mainly mitochondrial protein that forms cardiolipin- and prohibitin-enriched microdomains in the mitochondrial inner membrane that are important for the formation of respiratory supercomplexes and their function...
2017: PloS One
https://www.readbyqxmd.com/read/28637784/tnfr2-stimulation-promotes-mitochondrial-fusion-via-stat3-and-nf-kb-dependent-activation-of-opa1-expression
#13
Jinliang Nan, Hengxun Hu, Yong Sun, Lianlian Zhu, Yingchao Wang, Zhiwei Zhong, Jing Zhao, Na Zhang, Ya Wang, Yaping Wang, Jian Ye, Ling Zhang, Xinyang Hu, Wei Zhu, Jian'an Wang
RATIONALE: Mitochondria are important cellular organelles and play essential roles in maintaining cell structure and function. Emerging evidence indicates that in addition to having proinflammatory and proapoptotic effects, TNFα (tumor necrosis factor α) can, under certain circumstances, promote improvements in mitochondrial integrity and function, phenomena that can be ascribed to the existence of TNFR2 (TNFα receptor 2). OBJECTIVE: The present study aimed to investigate whether and how TNFR2 activation mediates the effects of TNFα on mitochondria...
August 4, 2017: Circulation Research
https://www.readbyqxmd.com/read/28630166/deficiency-of-phb-complex-impairs-respiratory-supercomplex-formation-and-activates-mitochondrial-flashes
#14
Chongshu Jian, Fengli Xu, Tingting Hou, Tao Sun, Li Jinghang, Heping Cheng, Xianhua Wang
Prohibitins (prohibitin 1, PHB1, and prohibitin 2, PHB2) are evolutionally conserved and ubiquitously expressed mitochondrial protein. PHBs form multimeric ring complexes acting as scaffolds in the inner mitochondrial membrane. Mitochondrial flashes (mitoflashes) are newly discovered mitochondrial signaling events which reflect electrical and chemical excitations of the organelle. Here we investigate possible roles of PHBs in the regulation of mitoflash signaling. Down-regulation of PHBs increases mitoflash frequency by up to 5...
June 19, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28605384/analyzing-supercomplexes-of-the-mitochondrial-electron-transport-chain-with-native-electrophoresis-in-gel-assays-and-electroelution
#15
Gisela Beutner, George Arthur Porter
The mitochondrial electron transport chain (ETC) transduces the energy derived from the breakdown of various fuels into the bioenergetic currency of the cell, ATP. The ETC is composed of 5 massive protein complexes, which also assemble into supercomplexes called respirasomes (C-I, C-III, and C-IV) and synthasomes (C-V) that increase the efficiency of electron transport and ATP production. Various methods have been used for over 50 years to measure ETC function, but these protocols do not provide information on the assembly of individual complexes and supercomplexes...
June 1, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28593021/tissue-and-condition-specific-isoforms-of-mammalian-cytochrome-c-oxidase-subunits-from-function-to-human-disease
#16
REVIEW
Christopher A Sinkler, Hasini Kalpage, Joseph Shay, Icksoo Lee, Moh H Malek, Lawrence I Grossman, Maik Hüttemann
Cytochrome c oxidase (COX) is the terminal enzyme of the electron transport chain and catalyzes the transfer of electrons from cytochrome c to oxygen. COX consists of 14 subunits, three and eleven encoded, respectively, by the mitochondrial and nuclear DNA. Tissue- and condition-specific isoforms have only been reported for COX but not for the other oxidative phosphorylation complexes, suggesting a fundamental requirement to fine-tune and regulate the essentially irreversible reaction catalyzed by COX. This article briefly discusses the assembly of COX in mammals and then reviews the functions of the six nuclear-encoded COX subunits that are expressed as isoforms in specialized tissues including those of the liver, heart and skeletal muscle, lung, and testes: COX IV-1, COX IV-2, NDUFA4, NDUFA4L2, COX VIaL, COX VIaH, COX VIb-1, COX VIb-2, COX VIIaH, COX VIIaL, COX VIIaR, COX VIIIH/L, and COX VIII-3...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28584055/prolonged-fasting-suppresses-mitochondrial-nlrp3-inflammasome-assembly-and-activation-via-sirt3-mediated-activation-of-superoxide-dismutase-2
#17
COMPARATIVE STUDY
Javier Traba, Sarah S Geiger, Miriam Kwarteng-Siaw, Kim Han, One Hyuk Ra, Richard M Siegel, David Gius, Michael N Sack
Twenty-four hours of fasting is known to blunt activation of the human NLRP3 inflammasome. This effect might be mediated by SIRT3 activation, controlling mitochondrial reactive oxygen species. To characterize the molecular underpinnings of this fasting effect, we comparatively analyzed the NLRP3 inflammasome response to nutrient deprivation in wild-type and SIRT3 knock-out mice. Consistent with previous findings for human NLRP3, prolonged fasting blunted the inflammasome in wild-type mice but not in SIRT3 knock-out mice...
July 21, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28582861/post-translational-oxidative-modifications-of-mitochondrial-complex-i-nadh-ubiquinone-oxidoreductase-implications-for-pathogenesis-and-therapeutics-in-human-diseases
#18
M M Srinivas Bharath
Mitochondrial complex I (NADH: ubiquinone oxidoreductase; CI) is central to the electron transfer chain (ETC), oxidative phosphorylation, and ATP production in eukaryotes. CI is a multi-subunit complex with a complicated yet organized structure that optimally connects electron transfer with proton translocation and forms higher-order supercomplexes with other ETC complexes. Efforts to understand the molecular genetics, expression profile of subunits, and structure-function relationship of CI have increased over the years due to the direct role of the complex in human diseases...
May 30, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28579242/mitochonic-acid-5-ma-5-facilitates-atp-synthase-oligomerization-and-cell-survival-in-various-mitochondrial-diseases
#19
Tetsuro Matsuhashi, Takeya Sato, Shin-Ichiro Kanno, Takehiro Suzuki, Akihiro Matsuo, Yuki Oba, Motoi Kikusato, Emi Ogasawara, Tai Kudo, Kosuke Suzuki, Osamu Ohara, Hiroko Shimbo, Fumika Nanto, Hiroaki Yamaguchi, Daisuke Saigusa, Yasuno Mukaiyama, Akiko Watabe, Koichi Kikuchi, Hisato Shima, Eikan Mishima, Yasutoshi Akiyama, Yoshitsugu Oikawa, H O Hsin-Jung, Yukako Akiyama, Chitose Suzuki, Mitsugu Uematsu, Masaki Ogata, Naonori Kumagai, Masaaki Toyomizu, Atsushi Hozawa, Nariyasu Mano, Yuji Owada, Setsuya Aiba, Teruyuki Yanagisawa, Yoshihisa Tomioka, Shigeo Kure, Sadayoshi Ito, Kazuto Nakada, Ken-Ichiro Hayashi, Hitoshi Osaka, Takaaki Abe
Mitochondrial dysfunction increases oxidative stress and depletes ATP in a variety of disorders. Several antioxidant therapies and drugs affecting mitochondrial biogenesis are undergoing investigation, although not all of them have demonstrated favorable effects in the clinic. We recently reported a therapeutic mitochondrial drug mitochonic acid MA-5 (Tohoku J. Exp. Med., 2015). MA-5 increased ATP, rescued mitochondrial disease fibroblasts and prolonged the life span of the disease model "Mitomouse" (JASN, 2016)...
June 2017: EBioMedicine
https://www.readbyqxmd.com/read/28533780/innate-immune-function-of-mitochondrial-metabolism
#20
REVIEW
David Sancho, Michel Enamorado, Johan Garaude
Sensing of microbe-associated molecular patterns or danger signals by innate immune receptors drives a complex exchange of information. Innate receptor signaling not only triggers transcriptional events but also induces profound changes in metabolic fluxes, redox balance, and metabolite abundance thereby influencing immune cell function. Mitochondria are at the core of metabolic adaptation to the changing environment. The close interaction between mitochondrial metabolism and immune signaling has emerged as a central regulator of innate sensing...
2017: Frontiers in Immunology
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