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Tanja Hering, Kerstin Kojer, Nathalie Birth, Jaqueline Hallitsch, Jan-Willem Taanman, Michael Orth
There is evidence of an imbalance of mitochondrial fission and fusion in patients with Huntington's disease (HD) and HD animal models. Fission and fusion are important for mitochondrial homeostasis including mitochondrial DNA (mtDNA) maintenance and may be relevant for the selective striatal mtDNA depletion that we observed in the R6/2 fragment HD mouse model. We aimed to investigate the fission/fusion balance and the integrity of the mitochondrial membrane system in cortex and striatum of end-stage R6/2 mice and wild-type animals...
February 2017: Experimental Neurology
Alejandro Alvarado-Delgado, Guillermo Perales Ortiz, Ángel T Tello-López, Sergio Encarnación, Renaud Conde, Ángel G Martínez-Batallar, Ken Moran-Francia, Humberto Lanz-Mendoza
BACKGROUND: The behaviour of Anopheles spp. mosquitoes, vectors for Plasmodium parasites, plays a crucial role in the propagation of malaria to humans. Consequently, it is important to understand how the behaviour of these mosquitoes is influenced by the interaction between the brain and immunological status. The nervous system is intimately linked to the immune and endocrine systems. There is evidence that the malaria parasite alters the function of these systems upon infecting the mosquito...
October 11, 2016: Parasites & Vectors
Shiori Akabane, Midori Uno, Naoki Tani, Shunta Shimazaki, Natsumi Ebara, Hiroki Kato, Hidetaka Kosako, Toshihiko Oka
A mitochondrial kinase, PTEN-induced putative kinase 1 (PINK1), selectively recruits the ubiquitin ligase Parkin to damaged mitochondria, which modifies mitochondria by polyubiquitination, leading to mitochondrial autophagy. Here, we report that treatment with an adenylate cyclase agonist or expression of protein kinase A (PKA) impairs Parkin recruitment to damaged mitochondria and decreases PINK1 protein levels. We identified a mitochondrial membrane protein, MIC60 (also known as mitofilin), as a PKA substrate...
May 5, 2016: Molecular Cell
Victor S Van Laar, Sarah B Berman, Teresa G Hastings
Mitochondrial dysfunction has been implicated in Parkinson's disease (PD) neuropathology. Mic60, also known as mitofilin, is a protein of the inner mitochondrial membrane and a key component of the mitochondrial contact site and cristae junction organizing system (MICOS). Mic60 is critical for maintaining mitochondrial membrane structure and function. We previously demonstrated that mitochondrial Mic60 protein is susceptible to both covalent modification and loss in abundance following exposure to dopamine quinone...
July 2016: Neurobiology of Disease
Lydie Plecitá-Hlavatá, Hana Engstová, Lukáš Alán, Tomáš Špaček, Andrea Dlasková, Katarína Smolková, Jitka Špačková, Jan Tauber, Vendula Strádalová, Jan Malínský, Mark Lessard, Joerg Bewersdorf, Petr Ježek
The relationship of the inner mitochondrial membrane (IMM) cristae structure and intracristal space (ICS) to oxidative phosphorylation (oxphos) is not well understood. Mitofilin (subunit Mic60) of the mitochondrial contact site and cristae organizing system (MICOS) IMM complex is attached to the outer membrane (OMM) via the sorting and assembly machinery/topogenesis of mitochondrial outer membrane β-barrel proteins (SAM/TOB) complex and controls the shape of the cristae. ATP synthase dimers determine sharp cristae edges, whereas trimeric OPA1 tightens ICS outlets...
May 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
Takehiro Suzuki, Hiroaki Yamaguchi, Motoi Kikusato, Osamu Hashizume, Satoru Nagatoishi, Akihiro Matsuo, Takeya Sato, Tai Kudo, Tetsuro Matsuhashi, Kazutaka Murayama, Yuki Ohba, Shun Watanabe, Shin-Ichiro Kanno, Daichi Minaki, Daisuke Saigusa, Hiroko Shinbo, Nobuyoshi Mori, Akinori Yuri, Miyuki Yokoro, Eikan Mishima, Hisato Shima, Yasutoshi Akiyama, Yoichi Takeuchi, Koichi Kikuchi, Takafumi Toyohara, Chitose Suzuki, Takaharu Ichimura, Jun-Ichi Anzai, Masahiro Kohzuki, Nariyasu Mano, Shigeo Kure, Teruyuki Yanagisawa, Yoshihisa Tomioka, Masaaki Toyomizu, Kohei Tsumoto, Kazuto Nakada, Joseph V Bonventre, Sadayoshi Ito, Hitoshi Osaka, Ken-Ichi Hayashi, Takaaki Abe
Mitochondrial dysfunction causes increased oxidative stress and depletion of ATP, which are involved in the etiology of a variety of renal diseases, such as CKD, AKI, and steroid-resistant nephrotic syndrome. Antioxidant therapies are being investigated, but clinical outcomes have yet to be determined. Recently, we reported that a newly synthesized indole derivative, mitochonic acid 5 (MA-5), increases cellular ATP level and survival of fibroblasts from patients with mitochondrial disease. MA-5 modulates mitochondrial ATP synthesis independently of oxidative phosphorylation and the electron transport chain...
July 2016: Journal of the American Society of Nephrology: JASN
Chengli Ding, Zhifei Wu, Lei Huang, Yajie Wang, Jie Xue, Si Chen, Zixin Deng, Lianrong Wang, Zhiyin Song, Shi Chen
The inner mitochondrial membrane (IMM) invaginates to form cristae and the maintenance of cristae depends on the mitochondrial contact site (MICOS) complex. Mitofilin and CHCHD6, which physically interact, are two components of the MICOS. In this study, we performed immunoprecipitation experiments with Mitofilin and CHCHD6 antibodies and identified a complex containing Mitofilin, Sam50, and CHCHD 3 and 6. Using transcription activator-like effector nucleases (TALENs), we generated knockdown/knockout clones of Mitofilin and CHCHD6...
November 4, 2015: Scientific Reports
H Li, Y Ruan, K Zhang, F Jian, C Hu, L Miao, L Gong, L Sun, X Zhang, S Chen, H Chen, D Liu, Z Song
The MICOS complex (mitochondrial contact site and cristae organizing system) is essential for mitochondrial inner membrane organization and mitochondrial membrane contacts, however, the molecular regulation of MICOS assembly and the physiological functions of MICOS in mammals remain obscure. Here, we report that Mic60/Mitofilin has a critical role in the MICOS assembly, which determines the mitochondrial morphology and mitochondrial DNA (mtDNA) organization. The downregulation of Mic60/Mitofilin or Mic19/CHCHD3 results in instability of other MICOS components, disassembly of MICOS complex and disorganized mitochondrial cristae...
March 2016: Cell Death and Differentiation
Peter W Lindinger, Martine Christe, Alex N Eberle, Beatrice Kern, Ralph Peterli, Thomas Peters, Kamburapola J I Jayawardene, Ian M Fearnley, John E Walker
Obesity is associated with impaired mitochondrial function. This study compares mitochondrial protein expression in omental fat in obese and non-obese humans. Omental adipose tissue was obtained by surgical biopsy, adipocytes were purified and mitochondria isolated. Using anion-exchange chromatography, SDS-PAGE and mass-spectrometry, 128 proteins with potentially different abundances in patient groups were identified, 62 of the 128 proteins are mainly localized in the mitochondria. Further quantification of 12 of these 62 proteins by immune dot blot analysis revealed four proteins citrate synthase, HADHA, LETM1 and mitofilin being inversely associated with BMI, and mitofilin being inversely correlated with gender...
September 2015: Data in Brief
Alexander J Abrams, Robert B Hufnagel, Adriana Rebelo, Claudia Zanna, Neville Patel, Michael A Gonzalez, Ion J Campeanu, Laurie B Griffin, Saskia Groenewald, Alleene V Strickland, Feifei Tao, Fiorella Speziani, Lisa Abreu, Rebecca Schüle, Leonardo Caporali, Chiara La Morgia, Alessandra Maresca, Rocco Liguori, Raffaele Lodi, Zubair M Ahmed, Kristen L Sund, Xinjian Wang, Laura A Krueger, Yanyan Peng, Carlos E Prada, Cynthia A Prows, Elizabeth K Schorry, Anthony Antonellis, Holly H Zimmerman, Omar A Abdul-Rahman, Yaping Yang, Susan M Downes, Jeffery Prince, Flavia Fontanesi, Antonio Barrientos, Andrea H Németh, Valerio Carelli, Taosheng Huang, Stephan Zuchner, Julia E Dallman
Dominant optic atrophy (DOA) and axonal peripheral neuropathy (Charcot-Marie-Tooth type 2, or CMT2) are hereditary neurodegenerative disorders most commonly caused by mutations in the canonical mitochondrial fusion genes OPA1 and MFN2, respectively. In yeast, homologs of OPA1 (Mgm1) and MFN2 (Fzo1) work in concert with Ugo1, for which no human equivalent has been identified thus far. By whole-exome sequencing of patients with optic atrophy and CMT2, we identified four families with recessive mutations in SLC25A46...
August 2015: Nature Genetics
Peter W Lindinger, Martine Christe, Alex N Eberle, Beatrice Kern, Ralph Peterli, Thomas Peters, Kamburapola J I Jayawardene, Ian M Fearnley, John E Walker
UNLABELLED: Impaired mitochondrial function is important in obesity and the development of insulin resistance and diabetes. The aim of this study was to identify human adipocyte-derived mitochondrial proteins associated with obesity. Mitochondrial proteins from 20 abdominal omental adipose tissue biopsies (13 obese and 7 control subjects) were separated by anion-exchange chromatography coupled to SDS-PAGE. Protein contents were compared and identified by MALDI-TOF-TOF mass spectrometry...
June 21, 2015: Journal of Proteomics
Christine Ott, Eva Dorsch, Martin Fraunholz, Sebastian Straub, Vera Kozjak-Pavlovic
Mitochondrial inner membrane folds into cristae, which significantly increase its surface and are important for mitochondrial function. The stability of cristae depends on the mitochondrial contact site (MICOS) complex. In human mitochondria, the inner membrane MICOS complex interacts with the outer membrane sorting and assembly machinery (SAM) complex, to form the mitochondrial intermembrane space bridging complex (MIB). We have created knockdown cell lines of most of the MICOS and MIB components and have used them to study the importance of the individual subunits for the cristae formation and complex stability...
2015: PloS One
Hyo-In Hwang, Tae-Hyong Lee, Young-Joo Jang
Dental pulp is a soft tissue located inside the hard part of a tooth and it contains a stem cell population that can regenerate damaged dentin and/or pulp itself. Human dental pulp stem cells (hDPSCs) are multipotent adult stem cells that have the potential to be differentiated into a variety of cell types. Although cells cultured primarily from pulp tissue show heterogeneous phenotypes and variable efficiency in their dentinogenic differentiation, proper selection markers, which are specific to hDPSCs, are essential for the osteo/dentinogenic study of human dental pulp cells...
June 1, 2015: Stem Cells and Development
Matthew W Gorr, Loren E Wold
No abstract text is available yet for this article.
August 2015: Journal of Molecular and Cellular Cardiology
Dharendra Thapa, Cody E Nichols, Sara E Lewis, Danielle L Shepherd, Rajaganapathi Jagannathan, Tara L Croston, Kevin J Tveter, Anthony A Holden, Walter A Baseler, John M Hollander
Mitofilin, also known as heart muscle protein, is an inner mitochondrial membrane structural protein that plays a central role in maintaining cristae morphology and structure. It is a critical component of the mitochondrial contact site and cristae organizing system (MICOS) complex which is important for mitochondrial architecture and cristae morphology. Our laboratory has previously reported alterations in mitochondrial morphology and proteomic make-up during type 1 diabetes mellitus, with mitofilin being significantly down-regulated in interfibrillar mitochondria (IFM)...
February 2015: Journal of Molecular and Cellular Cardiology
Kelly K Andringa, Uduak S Udoh, Aimee Landar, Shannon M Bailey
Chronic ethanol-mediated oxidative stress and lipid peroxidation increases the levels of various reactive lipid species including 4-hydroxynonenal (4-HNE), which can subsequently modify proteins in the liver. It has been proposed that 4-HNE modification adversely affects the structure and/or function of mitochondrial proteins, thereby impairing mitochondrial metabolism. To determine whether chronic ethanol consumption increases levels of 4-HNE modified proteins in mitochondria, male rats were fed control and ethanol-containing diets for 5 weeks and mitochondrial samples were analyzed using complementary proteomic methods...
2014: Redox Biology
Silvia Ravera, Cristina Capanni, Danika Tognotti, Roberta Bottega, Marta Columbaro, Carlo Dufour, Enrico Cappelli, Paolo Degan
Bone marrow (BM) failure, increased risk of myelodysplastic syndrome, acute leukaemia and solid tumors, endocrinopathies and congenital abnormalities are the major clinical problems in Fanconi anemia patients (FA). Chromosome instability and DNA repair defects are the cellular characteristics used for the clinical diagnosis. However, these biological defects are not sufficient to explain all the clinical phenotype of FA patients. The known defects are structural alteration in cell cytoskeleton, altered structural organization for intermediate filaments, nuclear lamina, and mitochondria...
March 2015: Journal of Cellular Physiology
Aldo Moreno-Ulloa, Leonardo Nogueira, Alonso Rodriguez, Jonathan Barboza, Michael C Hogan, Guillermo Ceballos, Francisco Villarreal, Israel Ramirez-Sanchez
There is evidence implicating oxidative stress (OS) as the cause of the deleterious effects of aging. In this study, we evaluated the capacity of the flavanol (-)-epicatechin (Epi) to reduce aging-induced OS and restore mitochondrial biogenesis, as well as, structural and functional endpoints in aged mice. Senile (S; 26-month-old) C57BL/6 male mice were randomly assigned to receive either water (vehicle) or 1mg/kg of Epi via oral gavage (twice daily) for 15 days. Young (Y; 6-month-old) mice were used as controls...
November 2015: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
Fotis Ioakeimidis, Christine Ott, Vera Kozjak-Pavlovic, Foteini Violitzi, Vagelis Rinotas, Eleni Makrinou, Elias Eliopoulos, Costas Fasseas, George Kollias, Eleni Douni
Mitochondrial structure and function is emerging as a major contributor to neuromuscular disease, highlighting the need for the complete elucidation of the underlying molecular and pathophysiological mechanisms. Following a forward genetics approach with N-ethyl-N-nitrosourea (ENU)-mediated random mutagenesis, we identified a novel mouse model of autosomal recessive neuromuscular disease caused by a splice-site hypomorphic mutation in a novel gene of unknown function, DnaJC11. Recent findings have demonstrated that DNAJC11 protein co-immunoprecipitates with proteins of the mitochondrial contact site (MICOS) complex involved in the formation of mitochondrial cristae and cristae junctions...
2014: PloS One
Nikolaus Pfanner, Martin van der Laan, Paolo Amati, Roderick A Capaldi, Amy A Caudy, Agnieszka Chacinska, Manjula Darshi, Markus Deckers, Suzanne Hoppins, Tateo Icho, Stefan Jakobs, Jianguo Ji, Vera Kozjak-Pavlovic, Chris Meisinger, Paul R Odgren, Sang Ki Park, Peter Rehling, Andreas S Reichert, M Saeed Sheikh, Susan S Taylor, Nobuo Tsuchida, Alexander M van der Bliek, Ida J van der Klei, Jonathan S Weissman, Benedikt Westermann, Jiping Zha, Walter Neupert, Jodi Nunnari
The mitochondrial inner membrane contains a large protein complex that functions in inner membrane organization and formation of membrane contact sites. The complex was variably named the mitochondrial contact site complex, mitochondrial inner membrane organizing system, mitochondrial organizing structure, or Mitofilin/Fcj1 complex. To facilitate future studies, we propose to unify the nomenclature and term the complex "mitochondrial contact site and cristae organizing system" and its subunits Mic10 to Mic60...
March 31, 2014: Journal of Cell Biology
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