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Mitochondrial supercomplexes

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https://www.readbyqxmd.com/read/28936218/atoma1-affects-the-oxphos-system-and-plant-growth-in-contrast-to-other-newly-identified-atp-independent-proteases-in-arabidopsis-mitochondria
#1
Iwona Migdal, Renata Skibior-Blaszczyk, Malgorzata Heidorn-Czarna, Marta Kolodziejczak, Arnold Garbiec, Hanna Janska
Compared with yeast, our knowledge on members of the ATP-independent plant mitochondrial proteolytic machinery is rather poor. In the present study, using confocal microscopy and immunoblotting, we proved that homologs of yeast Oma1, Atp23, Imp1, Imp2, and Oct1 proteases are localized in Arabidopsis mitochondria. We characterized these components of the ATP-independent proteolytic system as well as the earlier identified protease, AtICP55, with an emphasis on their significance in plant growth and functionality in the OXPHOS system...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/28914882/sirt3-sod2-maintains-osteoblast-differentiation-and-bone-formation-by-regulating-mitochondrial-stress
#2
Jing Gao, Zhihui Feng, Xueqiang Wang, Mengqi Zeng, Jing Liu, Shujun Han, Jie Xu, Lei Chen, Ke Cao, Jiangang Long, Zongfang Li, Weili Shen, Jiankang Liu
Recent studies have revealed robust metabolic changes during cell differentiation. Mitochondria, the organelles where many vital metabolic reactions occur, may play an important role. Here, we report the involvement of SIRT3-regulated mitochondrial stress in osteoblast differentiation and bone formation. In both the osteoblast cell line MC3T3-E1 and primary calvarial osteoblasts, robust mitochondrial biogenesis and supercomplex formation were observed during differentiation, accompanied by increased ATP production and decreased mitochondrial stress...
September 15, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28888990/chemicals-or-mutations-that-target-mitochondrial-translation-can-rescue-the-respiratory-deficiency-of-yeast-bcs1-mutants
#3
C Panozzo, A Laleve, D Tribouillard-Tanvier, J Ostojić, C Sellem, G Friocourt, A Bourand-Plantefol, A Burg, A Delahodde, M Blondel, G Dujardin
Bcs1p is a chaperone that is required for the incorporation of the Rieske subunit within complex III of the mitochondrial respiratory chain. Mutations in the human gene BCS1L (BCS1-like) are the most frequent nuclear mutations resulting in complex III-related pathologies. In yeast, the mimicking of some pathogenic mutations causes a respiratory deficiency. We have screened chemical libraries and found that two antibiotics, pentamidine and clarithromycin, can compensate two bcs1 point mutations in yeast, one of which is the equivalent of a mutation found in a human patient...
September 6, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28870773/mitochondrial-cytochrome-c-oxidase-biogenesis-recent-developments
#4
REVIEW
Alba Timón-Gómez, Eva Nývltová, Luciano A Abriata, Alejandro J Vila, Jonathan Hosler, Antoni Barrientos
Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen consumption and is essential for aerobic energy generation in the form of ATP. Human COX is a copper-heme A hetero-multimeric complex formed by 3 catalytic core subunits encoded in the mitochondrial DNA and 11 subunits encoded in the nuclear genome. Investigations over the last 50 years have progressively shed light into the sophistication surrounding COX biogenesis and the regulation of this process, disclosing multiple assembly factors, several redox-regulated processes leading to metal co-factor insertion, regulatory mechanisms to couple synthesis of COX subunits to COX assembly, and the incorporation of COX into respiratory supercomplexes...
September 7, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28853723/ndufaf4-variants-are-associated-with-leigh-syndrome-and-cause-a-specific-mitochondrial-complex-i-assembly-defect
#5
Fabian Baertling, Laura Sánchez-Caballero, Mariël A M van den Brand, Liesbeth T Wintjes, Maaike Brink, Frans A van den Brandt, Callum Wilson, Richard J T Rodenburg, Leo G J Nijtmans
Mitochondrial respiratory chain complex I consists of 44 different subunits and can be subgrouped into three functional modules: the Q-, the P- and the N-module. NDUFAF4 (C6ORF66) is an assembly factor of complex I that associates with assembly intermediates of the Q-module. Via exome sequencing, we identified a homozygous missense variant in a complex I-deficient patient with Leigh syndrome. Supercomplex analysis in patient fibroblasts revealed specifically altered stoichiometry. Detailed assembly analysis of complex I, indicative of all of its assembly routes, showed an accumulation of parts of the P- and the N-module but not the Q-module...
August 30, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28844695/architecture-of-human-mitochondrial-respiratory-megacomplex-i2iii2iv2
#6
Runyu Guo, Shuai Zong, Meng Wu, Jinke Gu, Maojun Yang
The respiratory megacomplex represents the highest-order assembly of respiratory chain complexes, and it allows mitochondria to respond to energy-requiring conditions. To understand its architecture, we examined the human respiratory chain megacomplex-I2III2IV2 (MCI2III2IV2) with 140 subunits and a subset of associated cofactors using cryo-electron microscopy. The MCI2III2IV2 forms a circular structure with the dimeric CIII located in the center, where it is surrounded by two copies each of CI and CIV. Two cytochrome c (Cyt...
September 7, 2017: Cell
https://www.readbyqxmd.com/read/28821616/cox16-protein-is-physically-associated-with-cox1p-assembly-intermediates-and-with-cytochrome-oxidase
#7
Chen Hsien Su, Alexander Tzagoloff
Mitochondrial cytochrome oxidase (COX) catalyzes the last step in the respiratory pathway. In Saccharomyces cervisiae this inner membrane complex is composed of 11 protein subunits. Expression of COX is assisted by some 2 dozen ancillary proteins that intercede at different stages of the assembly pathway. One such protein, Cox16p, encoded by COX16, was shown to be essential for activity and assembly of COX. The function of Cox16p, however, has not been determined. We present evidence that Cox16p is present in Cox1p assembly intermediates and in COX...
August 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28804536/combined-respiratory-chain-deficiency-and-uqcc2-mutations-in-neonatal-encephalomyopathy-defective-supercomplex-assembly-in-complex-iii-deficiencies
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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 23, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28736227/association-between-ros-production-swelling-and-the-respirasome-integrity-in-cardiac-mitochondria
#16
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...
September 15, 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
#17
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
#18
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
#19
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
#20
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
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