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Mitochondrial supercomplexes

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https://www.readbyqxmd.com/read/29343773/mitochondrial-mutations-in-cholestatic-liver-disease-with-biliary-atresia
#1
Hong Koh, Gun-Seok Park, Sun-Mi Shin, Chang Eon Park, Seung Kim, Seok Joo Han, Huy Quang Pham, Jae-Ho Shin, Dong-Woo Lee
Biliary atresia (BA) results in severe bile blockage and is caused by the absence of extrahepatic ducts. Even after successful hepatic portoenterostomy, a considerable number of patients are likely to show progressive deterioration in liver function. Recent studies show that mutations in protein-coding mitochondrial DNA (mtDNA) genes and/or mitochondrial genes in nuclear DNA (nDNA) are associated with hepatocellular dysfunction. This observation led us to investigate whether hepatic dysfunctions in BA is genetically associated with mtDNA mutations...
January 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29343425/iba57p-participates-in-maturation-of-a-2fe-2s-cluster-rieske-protein-and-in-formation-of-supercomplexes-iii-iv-of-saccharomyces-cerevisiae-electron-transport-chain
#2
Luis A Sánchez, Mauricio Gomez-Gallardo, Alma L Díaz-Pérez, Christian Cortes-Rojo, Jesús Campos-García
The [Fe-S] late-acting subsystem comprised of Isa1p/Isa2p, Grx5p, and Iba57p proteins (Fe-S-IBG subsystem) is involved in [4Fe-4S]-cluster protein assembly. The effect of deleting IBA57 in Saccharomyces cerevisiae on mitochondrial respiratory complex integration and functionality associated with Rieske protein maturation was evaluated. The iba57Δ mutant showed decreased expression and maturation of the Rieske protein. The loss of Rieske protein caused by IBA57 deletion affected the structure of supercomplexes III2IV2 and III2IV1 and their integration into the mitochondria, causing dysfunction in the electron transport chain...
January 14, 2018: Mitochondrion
https://www.readbyqxmd.com/read/29317722/loss-of-the-mitochondrial-fatty-acid-%C3%AE-oxidation-protein-medium-chain-acyl-coenzyme-a-dehydrogenase-disrupts-oxidative-phosphorylation-protein-complex-stability-and-function
#3
Sze Chern Lim, Makiko Tajika, Masaru Shimura, Kirstyn T Carey, David A Stroud, Kei Murayama, Akira Ohtake, Matthew McKenzie
Medium-chain acyl-Coenzyme A dehydrogenase (MCAD) is involved in the initial step of mitochondrial fatty acid β-oxidation (FAO). Loss of function results in MCAD deficiency, a disorder that usually presents in childhood with hypoketotic hypoglycemia, vomiting and lethargy. While the disruption of mitochondrial fatty acid metabolism is the primary metabolic defect, secondary defects in mitochondrial oxidative phosphorylation (OXPHOS) may also contribute to disease pathogenesis. Therefore, we examined OXPHOS activity and stability in MCAD-deficient patient fibroblasts that have no detectable MCAD protein...
January 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29240987/nmr-study-of-rcf2-reveals-an-unusual-dimeric-topology-in-detergent-micelles
#4
Shu Zhou, Pontus Pettersson, Peter Brzezinski, Pia Ädelroth, Lena Mäler
The Saccharomyces (S.) cerevisiae mitochondrial respiratory supercomplex factor 2 (Rcf2) plays a role in assembly of supercomplexes composed of cytochrome bc1 (complex III) and cytochrome c oxidase (complex IV). The Rcf2 protein was here expressed in E. coli, refolded and reconstituted into dodecylphosphocholine (DPC) micelles. The structural properties of Rcf2 were studied by solution NMR and near complete backbone assignment of Rcf2 was achieved. The secondary structure of Rcf2 contains seven helices, of which five are putative transmembrane (TM) helices, including, unexpectedly, a region formed by a charged 20-residue helix at the C-terminus...
December 14, 2017: Chembiochem: a European Journal of Chemical Biology
https://www.readbyqxmd.com/read/29233977/the-mitochondrial-negative-regulator-mcj-is-a-therapeutic-target-for-acetaminophen-induced-liver-injury
#5
Lucía Barbier-Torres, Paula Iruzubieta, David Fernández-Ramos, Teresa C Delgado, Daniel Taibo, Virginia Guitiérrez-de-Juan, Marta Varela-Rey, Mikel Azkargorta, Nicolas Navasa, Pablo Fernández-Tussy, Imanol Zubiete-Franco, Jorge Simon, Fernando Lopitz-Otsoa, Sofia Lachiondo-Ortega, Javier Crespo, Steven Masson, Misti Vanette McCain, Erica Villa, Helen Reeves, Felix Elortza, Maria Isabel Lucena, Maria Isabel Hernández-Alvarez, Antonio Zorzano, Raúl J Andrade, Shelly C Lu, José M Mato, Juan Anguita, Mercedes Rincón, María Luz Martínez-Chantar
Acetaminophen (APAP) is the active component of many medications used to treat pain and fever worldwide. Its overuse provokes liver injury and it is the second most common cause of liver failure. Mitochondrial dysfunction contributes to APAP-induced liver injury but the mechanism by which APAP causes hepatocyte toxicity is not completely understood. Therefore, we lack efficient therapeutic strategies to treat this pathology. Here we show that APAP interferes with the formation of mitochondrial respiratory supercomplexes via the mitochondrial negative regulator MCJ, and leads to decreased production of ATP and increased generation of ROS...
December 12, 2017: Nature Communications
https://www.readbyqxmd.com/read/29222160/the-interactome-of-intact-mitochondria-by-cross-linking-mass-spectrometry-provides-evidence-for-co-existing-respiratory-supercomplexes
#6
Fan Liu, Philip Lössl, Beverley M Rabbitts, Robert S Balaban, Albert J R Heck
Mitochondria exert an immense amount of cytophysiological functions, but the structural basis of most of these processes is still poorly understood. Here we use cross-linking mass spectrometry to probe the organization of proteins in native mouse heart mitochondria. Our approach provides the largest survey of mitochondrial protein interactions reported so far. In total, we identify 3,322 unique residue-to-residue contacts involving half of the mitochondrial proteome detected by bottom-up proteomics. The obtained mitochondrial protein interactome gives insights in the architecture of defined protein assemblies, the sub-mitochondrial localization, and the mitochondrial localization of five proteins not yet included in the MitoCarta database...
December 8, 2017: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/29191512/a-unique-respiratory-adaptation-in-drosophila-independent-of-supercomplex-formation
#7
Satoru Shimada, Marika Oosaki, Ryoko Takahashi, Shigefumi Uene, Sachiko Yanagisawa, Tomitake Tsukihara, Kyoko Shinzawa-Itoh
Large assemblies of respiratory chain complexes, known as supercomplexes, are present in the mitochondrial membrane in mammals and yeast, as well as in some bacterial membranes. The formation of supercomplexes is thought to contribute to efficient electron transfer, stabilization of each enzyme complex, and inhibition of reactive oxygen species (ROS) generation. In this study, mitochondria from various organisms were solubilized with digitonin, and then the solubilized complexes were separated by blue native PAGE (BN-PAGE)...
November 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29162722/docosahexaenoic-acid-lowers-cardiac-mitochondrial-enzyme-activity-by-replacing-linoleic-acid-in-the-phospholipidome
#8
E Madison Sullivan, Edward Ross Pennington, Genevieve C Sparagna, Maria J Torres, P Darrell Neufer, Mitchel Harris, James Washington, Ethan J Anderson, Tonya N Zeczycki, David A Brown, Saame Raza Shaikh
Cardiac mitochondrial phospholipid acyl chains regulate respiratory enzymatic activity. In several diseases, the rodent cardiac phospholipidome is extensively rearranged; however, whether specific acyl chains impair respiratory enzyme function is unknown. One unique remodeling event in the myocardium of obese and diabetic rodents is an increase in docosahexaenoic acid (DHA) levels. Here, we first confirmed that cardiac DHA levels are elevated in diabetic humans relative to controls. We then used dietary supplementation of a Western diet with DHA as a tool to promote cardiac acyl chain remodeling and to study its influence on respiratory enzyme function...
November 21, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29103101/ischemic-preconditioning-protects-astrocytes-against-oxygen-glucose-deprivation-via-the-nuclear-erythroid-2-related-factor-2-pathway
#9
Srinivasan V Narayanan, Kunjan R Dave, Miguel A Perez-Pinzon
Induction of ischemic preconditioning (IPC) represents a potential therapy against cerebral ischemia by activation of adaptive pathways and modulation of mitochondria to induce ischemic tolerance to various cells and tissues. Mitochondrial dysfunction has been ascribed to contribute to numerous neurodegenerative conditions and cerebral ischemia. Nuclear erythroid 2-related factor 2 (Nrf2) is a transcription factor that has traditionally been involved in upregulating cellular antioxidant systems to combat oxidative stress in the brain; however, the association of Nrf2 with mitochondria in the brain remains unclear...
November 4, 2017: Translational Stroke Research
https://www.readbyqxmd.com/read/29067684/the-role-of-non-bilayer-phospholipids-in-mitochondrial-structure-and-function
#10
REVIEW
Writoban Basu Ball, John K Neff, Vishal M Gohil
Mitochondrial structure and function are influenced by the unique phospholipid composition of its membranes. While mitochondria contain all the major classes of phospholipids, recent studies have highlighted specific roles of the non-bilayer forming phospholipids phosphatidylethanolamine (PE) and cardiolipin (CL) in the assembly and activity of mitochondrial respiratory chain (MRC) complexes. The non-bilayer phospholipids are cone-shaped molecules that introduce curvature stress in the bilayer membrane and have been shown to impact mitochondrial fusion and fission...
October 25, 2017: FEBS Letters
https://www.readbyqxmd.com/read/29066618/epigenetic-modification-of-mir-663-controls-mitochondria-to-nucleus-retrograde-signaling-and-tumor-progression
#11
Trevor Carden, Bhupendra Singh, Ved Mooga, Prachi Bajpai, Keshav K Singh
The normal cellular function requires communication between mitochondria and the nucleus, termed mitochondria-to-nucleus retrograde signaling. Disruption of this mechanism has been implicated in the development of cancers. Many proteins are known modulators of retrograde signaling, but whether microRNAs (miRNAs) are also involved is unknown. We conducted an miRNA microarray analysis using RNA from a parental cell line, a Rho0 line lacking mitochondrial DNA (mtDNA) and a Rho0 line with restored mtDNA. We found that miR-663 was down-regulated in the mtDNA-depleted Rho0 line...
December 15, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29018038/increased-localization-of-app-c99-in-mitochondria-associated-er-membranes-causes-mitochondrial-dysfunction-in-alzheimer-disease
#12
Marta Pera, Delfina Larrea, Cristina Guardia-Laguarta, Jorge Montesinos, Kevin R Velasco, Rishi R Agrawal, Yimeng Xu, Robin B Chan, Gilbert Di Paolo, Mark F Mehler, Geoffrey S Perumal, Frank P Macaluso, Zachary Z Freyberg, Rebeca Acin-Perez, Jose Antonio Enriquez, Eric A Schon, Estela Area-Gomez
In the amyloidogenic pathway associated with Alzheimer disease (AD), the amyloid precursor protein (APP) is cleaved by β-secretase to generate a 99-aa C-terminal fragment (C99) that is then cleaved by γ-secretase to generate the β-amyloid (Aβ) found in senile plaques. In previous reports, we and others have shown that γ-secretase activity is enriched in mitochondria-associated endoplasmic reticulum (ER) membranes (MAM) and that ER-mitochondrial connectivity and MAM function are upregulated in AD We now show that C99, in addition to its localization in endosomes, can also be found in MAM, where it is normally processed rapidly by γ-secretase...
November 15, 2017: EMBO Journal
https://www.readbyqxmd.com/read/28988874/regulation-of-mammalian-13-subunit-cytochrome-c-oxidase-and-binding-of-other-proteins-role-of-ndufa4
#13
REVIEW
Bernhard Kadenbach
Cytochrome c oxidase (CcO) is the final oxygen accepting enzyme complex (complex IV) of the mitochondrial respiratory chain. In contrast to the other complexes (I, II, and III), CcO is highly regulated via isoforms for six of its ten nuclear-coded subunits, which are differentially expressed in species, tissues, developmental stages, and cellular oxygen concentrations. Recent publications have claimed that NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 4 (NDUFA4), originally identified as subunit of complex I, represents a 14th subunit of CcO...
November 2017: Trends in Endocrinology and Metabolism: TEM
https://www.readbyqxmd.com/read/28981073/clarifying-the-supercomplex-the-higher-order-organization-of-the-mitochondrial-electron-transport-chain
#14
REVIEW
James A Letts, Leonid A Sazanov
The oxidative phosphorylation electron transport chain (OXPHOS-ETC) of the inner mitochondrial membrane is composed of five large protein complexes, named CI-CV. These complexes convert energy from the food we eat into ATP, a small molecule used to power a multitude of essential reactions throughout the cell. OXPHOS-ETC complexes are organized into supercomplexes (SCs) of defined stoichiometry: CI forms a supercomplex with CIII2 and CIV (SC I+III2+IV, known as the respirasome), as well as with CIII2 alone (SC I+III2)...
October 5, 2017: Nature Structural & Molecular Biology
https://www.readbyqxmd.com/read/28967163/mild-phenotypes-and-proper-supercomplex-assembly-in-human-cells-carrying-the-homoplasmic-m-15557g%C3%A2-%C3%A2-a-mutation-in-cytochrome-b-gene
#15
Luisa Iommarini, Anna Ghelli, Giulia Leone, Concetta Valentina Tropeano, Ivana Kurelac, Laura Benedetta Amato, Giuseppe Gasparre, Anna Maria Porcelli
Respiratory complex III (CIII) is the first enzymatic bottleneck of the mitochondrial respiratory chain both in its native dimeric form and in supercomplexes. The mammalian CIII comprises 11 subunits among which cytochrome b is central in the catalytic core, where oxidation of ubiquinol occurs at the Qo site. The Qo- or PEWY-motif of cytochrome b is the most conserved through species. Importantly, the highly conserved glutamate at position 271 (Glu271) has never been studied in higher eukaryotes so far and its role in the Q-cycle remains debated...
October 1, 2017: Human Mutation
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
#16
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
#17
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...
February 2018: 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
#18
C Panozzo, A Laleve, D Tribouillard-Tanvier, J Ostojić, C H 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...
December 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28870773/mitochondrial-cytochrome-c-oxidase-biogenesis-recent-developments
#19
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
#20
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...
November 2017: European Journal of Human Genetics: EJHG
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