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https://www.readbyqxmd.com/read/28904209/drosophila-mic60-mitofilin-conducts-dual-roles-in-mitochondrial-motility-and-crista-structure
#1
Pei-I Tsai, Amanda M Papakyrikos, Chung-Han Hsieh, Xinnan Wang
MIC60/mitofilin constitutes a hetero-oligomeric complex on the inner mitochondrial membranes to maintain crista structure. However, little is known about its physiological functions. Here, by characterizing Drosophila MIC60 mutants, we define its roles in vivo We discover that MIC60 performs dual functions to maintain mitochondrial homeostasis. In addition to its canonical role in crista membrane structure, MIC60 regulates mitochondrial motility, likely by influencing protein levels of the outer mitochondrial membrane protein Miro that anchors mitochondria to the microtubule motors...
September 13, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28710113/trap1-ameliorates-renal-tubulointerstitial-fibrosis-in-mice-with-unilateral-ureteral-obstruction-by-protecting-renal-tubular-epithelial-cell-mitochondria
#2
Jun-Feng Chen, Qi-Shun Wu, Yu-Xian Xie, Bo-Lin Si, Ping-Ping Yang, Wen-Yan Wang, Qin Hua, Qing He
Mitochondrial dysfunction causes renal tubular epithelial cell injury and promotes cell apoptosis and renal tubulointerstitial fibrosis (TIF) progression. TNF receptor-associated protein 1 (TRAP1) is a molecular chaperone protein that is localized in mitochondria. It plays an important role in cell apoptosis; however, its functional mechanism in TIF remains unclear. In this study, we observed the effects of TRAP1 in renal tubular epithelial cell mitochondria in mice with unilateral ureteral obstruction and its function in cell apoptosis and TIF...
October 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28579242/mitochonic-acid-5-ma-5-facilitates-atp-synthase-oligomerization-and-cell-survival-in-various-mitochondrial-diseases
#3
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/28561061/regulated-membrane-remodeling-by-mic60-controls-formation-of-mitochondrial-crista-junctions
#4
Manuel Hessenberger, Ralf M Zerbes, Heike Rampelt, Séverine Kunz, Audrey H Xavier, Bettina Purfürst, Hauke Lilie, Nikolaus Pfanner, Martin van der Laan, Oliver Daumke
The mitochondrial contact site and cristae organizing system (MICOS) is crucial for the formation of crista junctions and mitochondrial inner membrane architecture. MICOS contains two core components. Mic10 shows membrane-bending activity, whereas Mic60 (mitofilin) forms contact sites between inner and outer membranes. Here we report that Mic60 deforms liposomes into thin membrane tubules and thus displays membrane-shaping activity. We identify a membrane-binding site in the soluble intermembrane space-exposed part of Mic60...
May 31, 2017: Nature Communications
https://www.readbyqxmd.com/read/27889468/mitochondrial-cristae-remodelling-is-associated-with-disrupted-opa1-oligomerisation-in-the-huntington-s-disease-r6-2-fragment-model
#5
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
https://www.readbyqxmd.com/read/27724938/infection-with-plasmodium-berghei-ookinetes-alters-protein-expression-in-the-brain-of-anopheles-albimanus-mosquitoes
#6
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
https://www.readbyqxmd.com/read/27153535/pka-regulates-pink1-stability-and-parkin-recruitment-to-damaged-mitochondria-through-phosphorylation-of-mic60
#7
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
https://www.readbyqxmd.com/read/27001148/mic60-mitofilin-overexpression-alters-mitochondrial-dynamics-and-attenuates-vulnerability-of-dopaminergic-cells-to-dopamine-and-rotenone
#8
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
https://www.readbyqxmd.com/read/26887443/hypoxic-hepg2-cell-adaptation-decreases-atp-synthase-dimers-and-atp-production-in-inflated-cristae-by-mitofilin-down-regulation-concomitant-to-micos-clustering
#9
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
https://www.readbyqxmd.com/read/26609120/mitochonic-acid-5-binds-mitochondria-and-ameliorates-renal-tubular-and-cardiac-myocyte-damage
#10
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
https://www.readbyqxmd.com/read/26530328/mitofilin-and-chchd6-physically-interact-with-sam50-to-sustain-cristae-structure
#11
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
https://www.readbyqxmd.com/read/26250910/mic60-mitofilin-determines-micos-assembly-essential-for-mitochondrial-dynamics-and-mtdna-nucleoid-organization
#12
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
https://www.readbyqxmd.com/read/26217759/important-mitochondrial-proteins-in-human-omental-adipose-tissue-show-reduced-expression-in-obesity
#13
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
https://www.readbyqxmd.com/read/26168012/mutations-in-slc25a46-encoding-a-ugo1-like-protein-cause-an-optic-atrophy-spectrum-disorder
#14
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
https://www.readbyqxmd.com/read/25865306/important-mitochondrial-proteins-in-human-omental-adipose-tissue-show-reduced-expression-in-obesity
#15
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
https://www.readbyqxmd.com/read/25781180/detailed-analysis-of-the-human-mitochondrial-contact-site-complex-indicate-a-hierarchy-of-subunits
#16
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
https://www.readbyqxmd.com/read/25590652/cell-proliferation-inducing-protein-52-mitofilin-is-a-surface-antigen-on-undifferentiated-human-dental-pulp-stem-cells
#17
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
https://www.readbyqxmd.com/read/25500008/mitofilin-key-factor-in-diabetic-cardiomyopathy
#18
EDITORIAL
Matthew W Gorr, Loren E Wold
No abstract text is available yet for this article.
August 2015: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/25463274/transgenic-overexpression-of-mitofilin-attenuates-diabetes-mellitus-associated-cardiac-and-mitochondria-dysfunction
#19
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
https://www.readbyqxmd.com/read/25454745/proteomic-analysis-of-4-hydroxynonenal-4-hne-modified-proteins-in-liver-mitochondria-from-chronic-ethanol-fed-rats
#20
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
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