Difference between revisions of "O2k-Publications: Mitochondrial disease"
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'''''Sort in ascending/descending order by a click on one of the small symbols in squares below'''''. | '''''Sort in ascending/descending order by a click on one of the small symbols in squares below'''''. | ||
Default sorting: chronological. Empty fields appear first in ascending order. | Default sorting: chronological. Empty fields appear first in ascending order. | ||
{{#ask:[[Category:Publications]] [[Instrument and method::Oxygraph-2k]] [[ | {{#ask:[[Category:Publications]] [[Instrument and method::Oxygraph-2k]] [[Diseases::Inherited]] OR [[Stress::Mitochondrial disease]] | ||
|?Was published in year=Year | |?Was published in year=Year | ||
|?Has title=Reference | |?Has title=Reference | ||
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== O2k-Publications: Mitochondrial Disease; Degenerative Disease and Defect - Abstracts == | == O2k-Publications: Mitochondrial Disease; Degenerative Disease and Defect - Abstracts == | ||
'''''Sort in ascending/descending order by a click on one of the small symbols in squares below'''''. | '''''Sort in ascending/descending order by a click on one of the small symbols in squares below'''''. | ||
{{#ask:[[Category:Abstracts]] [[Instrument and method::Oxygraph-2k]] [[ | {{#ask:[[Category:Abstracts]] [[Instrument and method::Oxygraph-2k]] [[Diseases::Inherited]] OR [[Stress::Mitochondrial disease]] | ||
|?Was submitted in year=Year | |?Was submitted in year=Year | ||
|?Has title=Reference | |?Has title=Reference |
Latest revision as of 16:38, 13 January 2023
O2k-Publications: Mitochondrial disease
Sort in ascending/descending order by a click on one of the small symbols in squares below. Default sorting: chronological. Empty fields appear first in ascending order.
Year | Reference | Organism | Tissue;cell | |
---|---|---|---|---|
Pegoraro 2024 Adv Drug Deliv Rev | 2024 | Pegoraro C, Domingo I, Conejos-Sánchez I, Vicent MJ (2024) Unlocking the mitochondria for nanomedicine-based treatments: overcoming biological barriers, improving designs, and selecting verification techniques. | ||
Meldau 2024 Mol Genet Metab Rep | 2024 | Meldau S, Ackermann S, Riordan G, van der Watt GF, Spencer C, Raga S, Khan K, Blackhurst DM, van der Westhuizen FH (2024) A novel mitochondrial DNA variant in MT-ND6: m.14430A>C p.(Trp82Gly) identified in a patient with Leigh syndrome and complex I deficiency. Mol Genet Metab Rep 39:101078. https://doi.org/10.1016/j.ymgmr.2024.101078 | Human | Fibroblast |
Xia 2024 Nat Metab | 2024 | Xia W, Veeragandham P, Cao Y, Yayun Xu Y, Rhyne TE, Qian J, Hung C-W, Zhao P, Jones Y, Gao H, Liddle C, Yu RT, Downes M, Evans RM, Rydén M, Wabitsch M, Wang Z, Hakozaki H, Schöneberg J, Reilly SM, Huang J, Saltiel AR (2024) Obesity causes mitochondrial fragmentation and dysfunction in white adipocytes due to RalA activation. Nat Metab 6:273–89. https://doi.org/10.1038/s42255-024-00978-0 | Human Mouse | Fat |
Airik 2023 Antioxidants (Basel) | 2023 | Airik M, Arbore H, Childs E, Huynh AB, Phua YL, Chen CW, Aird K, Bharathi S, Zhang B, Conlon P, Kmoch S, Kidd K, Bleyer AJ, Vockley J, Goetzman E, Wipf P, Airik R (2023) Mitochondrial ROS triggers KIN pathogenesis in FAN1-deficient kidneys. https://doi.org/10.3390/antiox12040900 | Human | Kidney |
Kutschka 2023 Basic Res Cardiol | 2023 | Kutschka I, Bertero E, Wasmus C, Xiao K, Yang L, Chen X, Oshima Y, Fischer M, Erk M, Arslan B, Alhasan L, Grosser D, Ermer KJ, Nickel A, Kohlhaas M, Eberl H, Rebs S, Streckfuss-Bömeke K, Schmitz W, Rehling P, Thum T, Higuchi T, Rabinowitz J, Maack C, Dudek J (2023) Activation of the integrated stress response rewires cardiac metabolism in Barth syndrome. https://doi.org/10.1007/s00395-023-01017-x | Mouse | Heart |
Janz 2023 Mol Metab | 2023 | Janz A, Walz K, Cirnu A, Surjanto J, Urlaub D, Leskien M, Kohlhaas M, Nickel A, Brand T, Nose N, Wörsdörfer P, Wagner N, Higuchi T, Maack C, Dudek J, Lorenz K, Klopocki E, Ergün S, Duff HJ, Gerull B (2023) Mutations in DNAJC19 cause altered mitochondrial structure and increased mitochondrial respiration in human iPSC-derived cardiomyocytes. https://doi.org/10.1016/j.molmet.2023.101859 | Human | HeLa |
Lee 2022 Hum Mol Genet | 2022 | Lee RG, Balasubramaniam S, Stentenbach M, Kralj T, McCubbin T, Padman B, Smith J, Riley LG, Priyadarshi A, Peng L, Nuske MR, Webster R, Peacock K, Roberts P, Stark Z, Lemire G, Ito YA; Care4Rare Canada Consortium, Boycott KM, Geraghty MT, van Klinken JB, Ferdinandusse S, Zhou Y, Walsh R, Marcellin E, Thorburn DR, Rosciolli T, Fletcher J, Rackham O, Vaz FM, Reid GE, Filipovska A (2022) Deleterious variants in CRLS1 lead to cardiolipin deficiency and cause an autosomal recessive multi-system mitochondrial disease. https://doi.org/10.1093/hmg/ddac040 | Human | Fibroblast |
Aasander Frostner 2022 MitoFit | 2022 | Åsander Frostner E, Simón Serrano S, Chamkha I, Donnelly E, Elmér E, Hansson MJ (2022) Towards a treatment for mitochondrial disease: current compounds in clinical development. https://doi.org/10.26124/mitofit:2022-0014 — 2022-06-28 published in Bioenerg Commun 2022.4. | Human | |
Zhao 2022 J Cell Sci | 2022 | Zhao Q, Yan S, Lu J, Parker DJ, Wu H, Sun Q, Crossman DK, Liu S, Wang Q, Sesaki H, Mitra K, Liu K, Jiao K (2022) Drp1 regulates transcription of ribosomal protein genes in embryonic hearts. https://doi.org/10.1242/jcs.258956 | Mouse | Heart |
Aasander Frostner 2022 BEC | 2022 | Åsander Frostner E, Simón Serrano S, Chamkha I, Donnelly E, Elmér E, Hansson MJ (2022) Towards a treatment for mitochondrial disease: current compounds in clinical development. Bioenerg Commun 2022.4. https://doi.org/10.26124/bec:2022-0004 | Human | |
Zhou 2022 J Hum Genet | 2022 | Zhou X, Lou X, Zhou Y, Xie Y, Han X, Dong Q, Ying X, Laurentinah MR, Zhang L, Chen Z, Li D, Fang H, Lyu J, Yang Y, Wang Y (2022) Novel biallelic mutations in TMEM126B cause splicing defects and lead to Leigh-like syndrome with severe complex I deficiency. https://doi.org/10.1038/s10038-022-01102-4 | Human | Lymphocyte |
Adant 2022 Mol Metab | 2022 | Adant I, Bird M, Decru B, Windmolders P, Wallays M, de Witte P, Rymen D, Witters P, Vermeersch P, Cassiman D, Ghesquière B (2022) Pyruvate and uridine rescue the metabolic profile of OXPHOS dysfunction. https://doi.org/10.1016/j.molmet.2022.101537 | Human | Fibroblast |
Belal 2022 Biomedicines | 2022 | Belal S, Goudenège D, Bocca C, Dumont F, Chao De La Barca JM, Desquiret-Dumas V, Gueguen N, Geffroy G, Benyahia R, Kane S, Khiati S, Bris C, Aranyi T, Stockholm D, Inisan A, Renaud A, Barth M, Simard G, Reynier P, Letournel F, Lenaers G, Bonneau D, Chevrollier A, Procaccio V (2022) Glutamate-induced deregulation of krebs cycle in mitochondrial encephalopathy lactic acidosis syndrome stroke-like episodes (MELAS) syndrome is alleviated by ketone body exposure. https://doi.org/10.3390/biomedicines10071665 | Human | Neuroblastoma |
Schober 2022 Hum Mol Genet | 2022 | Schober FA, Tang JX, Sergeant K, Moedas MF, Zierz CM, Moore D, Smith C, Lewis D, Guha N, Hopton S, Falkous G, Lam A, Pyle A, Poulton J, Gorman GS, Taylor RW, Freyer C, Wredenberg A (2022) Pathogenic SLC25A26 variants impair SAH transport activity causing mitochondrial disease. https://doi.org/10.1093/hmg/ddac002 | Mouse Drosophila | Fibroblast |
Spielmann 2022 Mamm Genome | 2022 | Spielmann N, Schenkl C, Komlódi T, da Silva-Buttkus P, Heyne E, Rohde J, Amarie OV, Rathkolb B, Gnaiger E, Doenst T, Fuchs H, Gailus-Durner V, de Angelis MH, Szibor M (2022) Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction. Mamm Genome 10.1007/s00335-022-09973-w | Mouse | Heart |
Munoz-Pujol 2022 Brain Pathol | 2022 | Muñoz-Pujol G, Ortigoza-Escobar JD, Paredes-Fuentes AJ, Jou C, Ugarteburu O, Gort L, Yubero D, García-Cazorla A, O'Callaghan M, Campistol J, Muchart J, Yépez VA, Gusic M, Gagneur J, Prokisch H, Artuch R, Ribes A, Urreizti R, Tort F (2022) Leigh syndrome is the main clinical characteristic of PTCD3 deficiency. https://doi.org/10.1111/bpa.13134 | Human | Fibroblast |
Inak 2021 Nat Commun | 2021 | Inak G, Rybak-Wolf A, Lisowski P, Pentimalli TM, Jüttner R, Glažar P, Uppal K, Bottani E, Brunetti D, Secker C, Zink A, Meierhofer D, Henke MT, Dey M, Ciptasari U, Mlody B, Hahn T, Berruezo-Llacuna M, Karaiskos N, Di Virgilio M, Mayr JA, Wortmann SB, Priller J, Gotthardt M, Jones DP, Mayatepek E, Stenzel W, Diecke S, Kühn R, Wanker EE, Rajewsky N, Schuelke M, Prigione A (2021) Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome. Nat Commun 12:1929. | Nervous system | |
Gueguen 2021 Methods Mol Biol | 2021 | Gueguen N, Lenaers G, Reynier P, Weissig V, Edeas M (2021) Mitochondrial dysfunction in mitochondrial medicine: current limitations, pitfalls, and tomorrow. https://doi.org/10.1007/978-1-0716-1266-8_1 | ||
Friederich 2021 Mol Genet Metab | 2021 | Friederich MW, Geddes GC, Wortmann SB, Punnoose A, Wartchow E, Knight KM, Prokisch H, Creadon-Swindell G, Mayr JA, Van Hove JLK (2021) Pathogenic variants in MRPL44 cause infantile cardiomyopathy due to a mitochondrial translation defect. Mol Genet Metab 133:362-71. | Human | Fibroblast |
Cretin 2021 EMBO Mol Med | 2021 | Cretin E, Lopes P, Vimont E, Tatsuta T, Langer T, Gazi A, Sachse M, Yu-Wai-Man P, Reynier P, Wai T (2021) High-throughput screening identifies suppressors of mitochondrial fragmentation in OPA1 fibroblasts. EMBO Mol Med 13:13579. | Mouse | Fibroblast |
Sun 2021 J Genet Genomics | 2021 | Sun Y, Wei X, Fang F, Shen Y, Wei H, Li J, Ye X, Zhan Y, Ye X, Liu X, Yang W, Li Y, Geng X, Huang X, Ruan Y, Qin Z, Yi S, Lyu J, Fang H, Yu Y (2021) HPDL deficiency causes a neuromuscular disease by impairing the mitochondrial respiration. J Genet Genomics 48:727-36. | Human | HeLa Lymphocyte |
Acin-Perez 2021 Life (Basel) | 2021 | Acin-Perez R, Benincá C, Shabane B, Shirihai OS, Stiles L (2021) Utilization of human samples for assessment of mitochondrial bioenergetics: gold standards, limitations, and future perspectives. Life (Basel) 11:949. doi: 10.3390/life11090949 | Human | Skeletal muscle Blood cells Fibroblast Platelet |
De Moura Alvorcem 2021 J Neurochem | 2021 | de Moura Alvorcem, L, Britto R, Cecatto C, Roginski AC, Rohden F, Scholl JN, Guma FCR, Figueiro F, Amaral AU, Zanatta G, Seminotti B, Wajner M, Leipnitz G. (2021) Ethylmalonic acid impairs bioenergetics by disturbing succinate and glutamate oxidation and induces mitochondrial permeability transition pore opening in rat cerebellum. J Neurochem 158(2): 262–81. | Rat | Nervous system |
Cecatto 2020 Toxicol In Vitro | 2020 | Cecatto C, Amaral AU, Roginski AC, Castilho RF, Wajner M (2020) Impairment of mitochondrial bioenergetics and permeability transition induction caused by major long-chain fatty acids accumulating in VLCAD deficiency in skeletal muscle as potential pathomechanisms of myopathy. Toxicol In Vitro 62:104665. | Rat | Skeletal muscle |
Kawamura 2021 Mol Ther Nucleic Acids | 2020 | Kawamura E, Maruyama M, Abe J, Sudo A, Takeda A, Takada S, Yokota T, Kinugawa S, Harashima H, Yamada Y (2021) Validation of gene therapy for mutant mitochondria by delivering mitochondrial RNA using a MITO-porter. Mol Ther Nucleic Acids 20:687-98. | Human | Fibroblast |
Ferreira 2020 PLoS Genet | 2020 | Ferreira N, Andoniou CE, Perks KL, Ermer JA, Rudler DL, Rossetti G, Periyakaruppiah A, Wong JKY, Rackham O, Noakes PG, Degli-Esposti MA, Filipovska A (2020) Murine cytomegalovirus infection exacerbates complex IV deficiency in a model of mitochondrial disease. PLoS Genet 16:e1008604. | Mouse | Liver |
Wei 2020 Hum Mutat | 2020 | Wei X, Du M, Li D, Wen S, Xie J, Li Y, Chen A, Zhang K, Xu P, Jia M, Wen C, Zhou H, Lyu J, Yang Y, Fang H (2020) Mutations in FASTKD2 are associated with mitochondrial disease with multi-OXPHOS deficiency. Hum Mutat 41:961-72. | Human | Lymphocyte |
Chen 2020 Mol Genet Genomic Med | 2020 | Chen D, Zhao Q, Xiong J, Lou X, Han Q, Wei X, Xie J, Li X, Zhou H, Shen L, Yang Y, Fang H, Lyu J (2020) Systematic analysis of a mitochondrial disease-causing ND6 mutation in mitochondrial deficiency. Mol Genet Genomic Med 8:e1199. | Human | Lymphocyte |
Purhonen 2020 Nat Commun | 2020 | Purhonen J, Grigorjev V, Ekiert R, Aho N, Rajendran J, Pietras R, Truvé K, Wikström M, Sharma V, Osyczka A, Fellman V, Kallijärvi J (2020) A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice. Nat Commun 11:322. | Mouse | Liver Kidney |
Roginski 2020 Biochim Biophys Acta Mol Basis Dis | 2020 | Roginski AC, Wajner A, Cecatto C, Wajner SM, Castilho RF, Wajner M, Amaral AU (2020) Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats. Biochim Biophys Acta Mol Basis Dis 1866:165682. | Rat | Heart Other cell lines |
Burgin 2020 Int J Mol Sci | 2020 | Burgin HJ, Lopez Sanchez MIG, Smith CM, Trounce IA, McKenzie M (2020) Pioglitazone and deoxyribonucleoside combination treatment increases mitochondrial respiratory capacity in m.3243A>G MELAS cybrid cells. Int J Mol Sci 21:E2139. | Human | Other cell lines |
Cecatto 2020 Mitochondrion | 2020 | Cecatto C, Amaral AU, Wajner A, Wajner SM, Castilho RF, Wajner M (2020) Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats. Mitochondrion 50:1-13. | Rat | Liver Other cell lines |
Hassan 2020 MitoFit Preprint Arch | 2020 | Hassan Hazirah, Gnaiger Erich, Zakaria Fazaine, Makpol Suzana, Abdul Karim Norwahidah (2020) Alterations in mitochondrial respiratory capacity and membrane potential: a link between mitochondrial dysregulation and autism. https://doi.org/10.26124/mitofit:200003 | Human | |
Frambach 2020 Biochim Biophys Acta Mol Basis Dis | 2020 | Frambach SJCM, van de Wal MAE, van den Broek PHH, Smeitink JAM, Russel FGM, de Haas R, Schirris TJJ (2020) Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice. Biochim Biophys Acta Mol Basis Dis 1866:165727. | Mouse | Skeletal muscle |
Tepp 2020 Biochim Biophys Acta Gen Subj | 2020 | Tepp K, Puurand M, Timohhina N, Aid-Vanakova J, Reile I, Shevchuk I, Chekulayev V, Eimre M, Peet N, Kadaja L, Paju K, Kaambre T (2020) Adaptation of striated muscles to Wolframin deficiency in mice: Alterations in cellular bioenergetics. Biochim Biophys Acta Gen Subj 1864:129523. | Mouse | Heart Skeletal muscle |
Gaudo 2019 Neurogenetics | 2019 | Gaudó P, Emperador S, Garrido-Pérez N, Ruiz-Pesini E, Yubero D, García-Cazorla A, Artuch R, Montoya J, Bayona-Bafaluy MP (2019) Infectious stress triggers a POLG-related mitochondrial disease. Neurogenetics 21:19-27. | Human | Fibroblast |
Li 2019 Int Urol Nephrol | 2019 | Li Z, Zhou J, Li Y, Yang F, Lian X, Liu W (2019) Mitochondrial TRPC3 promotes cell proliferation by regulating the mitochondrial calcium and metabolism in renal polycystin-2 knockdown cells. Int Urol Nephrol 51:1059-70. | Human | Endothelial;epithelial;mesothelial cell |
Wolf 2019 Cells | 2019 | Wolf C, Zimmermann R, Thaher O, Bueno D, Wüllner V, Schäfer MKE, Albrecht P, Methner A (2019) The Charcot-Marie tooth disease mutation R94Q in MFN2 decreases ATP production but increases mitochondrial respiration under conditions of mild oxidative stress. Cells 8:E1289. | Mouse | Fibroblast |
Rajendran 2019 EMBO Mol Med | 2019 | Rajendran J, Purhonen J, Tegelberg S, Smolander OP, Mörgelin M, Rozman J, Gailus-Durner V, Fuchs H, Hrabe de Angelis M, Auvinen P, Mervaala E, Jacobs HT, Szibor M, Fellman V, Kallijärvi J (2019) Alternative oxidase-mediated respiration prevents lethal mitochondrial cardiomyopathy. EMBO Mol Med 11:e9456. | Mouse | Liver Kidney Heart |
Itkis 2019 Mitochondrion | 2019 | Itkis Y, Krylova T, Pechatnikova NL, De Grassi A, Tabakov VY, Pierri CL, Aleshin V, Boyko A, Bunik VI, Zakharova EY (2019) A novel variant m.641A>T in the mitochondrial MT-TF gene is associated with epileptic encephalopathy in adolescent. Mitochondrion 47:10-17. | Human | Fibroblast |
Germain 2019 Int J Med Sci | 2019 | Germain N, Dessein AF, Vienne JC, Dobbelaere D, Mention K, Joncquel M, Dekiouk S, Laine W, Kluza J, Marchetti P (2019) First-line screening of OXPHOS deficiencies using microscale oxygraphy in human skin fibroblasts: A preliminary study. Int J Med Sci 16:931-38. | Human | Fibroblast |
Repp 2019 Thesis | 2019 | Repp BM (2019) Identification and verification of novel disease-causing genes and therapy options for patients with mitochondrial disorders – Focus on ACAD9. PhD Thesis 178. | Human | HEK |
Friederich 2019 Mol Genet Metab | 2019 | Friederich MW, Perez FA, Knight KM, Van Hove RA, Yang SP, Saneto RP, Van Hove JLK (2019) Pathogenic variants in NUBPL result in failure to assemble the matrix arm of complex I and cause a complex leukoencephalopathy with thalamic involvement. Mol Genet Metab 129:236-42. | Human | Fibroblast |
Chen 2018 Hum Mol Genet | 2018 | Chen A, Tiosano D, Guran T, Baris HN, Bayram Y, Mory A, Shapiro-Kulnane L, Hodges CA, Coban Akdemir Z, Turan S, Jhangiani SN, van den Akker F, Hoppel CL, Salz HK, Lupski JR, Buchner DA (2018) Mutations in the mitochondrial ribosomal protein MRPS22 lead to primary ovarian insufficiency. Hum Mol Genet 27:1913-26. | Human | Endothelial;epithelial;mesothelial cell Fibroblast |
Sprenger 2018 EMBO Mol Med | 2018 | Sprenger HG, Wani G, Hesseling A, König T, Patron M, MacVicar T, Ahola S, Wai T, Barth E, Rugarli EI, Bergami M, Langer T (2018) Loss of the mitochondrial i-AAA protease YME1L leads to ocular dysfunction and spinal axonopathy. EMBO Mol Med 11:e9288. | Mouse | Nervous system |
Boutoual 2018 Scientific Reports | 2018 | Boutoual, R., Meseguer, S., Villarroya, M., Martin-Hernandez, E., Errami, M., Martin, M. A., Casado, M., and Armengod, M. E. (2018) Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. Scientific reports 8, 1163 | Human | Other cell lines Fibroblast |
Cecatto 2018 FEBS J | 2018 | Cecatto C, Amaral AU, da Silva JC, Wajner A, Schimit MOV, da Silva LHR, Wajner SM, Zanatta A, Castilho RF, Wajner M (2018) Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca2+ homeostasis in the heart. FEBS J 285:1437-55. | Rat | Heart Other cell lines |
Bouwkamp 2018 Neurol Genet | 2018 | Bouwkamp CG, Afawi Z, Fattal-Valevski A, Krabbendam IE, Rivetti S, Masalha R, Quadri M, Breedveld GJ, Mandel H, Tailakh MA, Beverloo HB, Stevanin G, Brice A, van IJcken WFJ, Vernooij MW, Dolga AM, de Vrij FMS, Bonifati V, Kushner SA (2018) ACO2 homozygous missense mutation associated with complicated hereditary spastic paraplegia. Neurol Genet 4:e223. | Human | |
Kauppila 2018 Thesis | 2018 | Kauppila JHK (2018) Generating mammalian mitochondrial disease models with mitochondrial DNA mutations. PhD Thesis p120. | Mouse | Heart Liver |
Li 2018 Gene | 2018 | Li Y, Wen S, Li D, Xie J, Wei X, Li X, Liu Y, Fang H, Yang Y, Lyu J (2018) SURF1 mutations in Chinese patients with Leigh syndrome: Novel mutations, mutation spectrum, and the functional consequences. Gene 674:15-24. | Human | Blood cells |
Ignatenko 2018 Nat Commun | 2018 | Ignatenko O, Chilov D, Paetau I, de Miguel E, Jackson CB, Capin G, Paetau A, Terzioglu M, Euro L, Suomalainen A (2018) Loss of mtDNA activates astrocytes and leads to spongiotic encephalopathy. Nat Commun 9:70. | Mouse | Nervous system |
Dogan 2018 Cell Metab | 2018 | Dogan SA, Cerutti R, Benincá C, Brea-Calvo G, Jacobs HT, Zeviani M, Szibor M, Viscomi C (2018) Perturbed redox signaling exacerbates a mitochondrial myopathy. Cell Metab 28:764-77. | Mouse | Skeletal muscle |
Harmuth 2018 Front Mol Neurosci | 2018 | Harmuth T, Prell-Schicker C, Weber JJ, Gellerich F, Funke C, Drießen S, Magg JCD, Krebiehl G, Wolburg H, Hayer SN, Hauser S, Krüger R, Schöls L, Riess O, Hübener-Schmid J (2018) Mitochondrial morphology, function and homeostasis are impaired by expression of an N-terminal calpain cleavage fragment of Ataxin-3. Front Mol Neurosci 11:368. | Mouse | Fibroblast |
Jia 2018 Circ Res | 2018 | Jia G, Hill MA, Sowers JR (2018) Diabetic cardiomyopathy: an update of mechanisms contributing to this clinical entity. Circ Res 122:624–38. | Human | Heart |
Angebault 2018 Sci Signal | 2018 | Angebault C, Fauconnier J, Patergnani S, Rieusset J, Danese A, Affortit CA, Jagodzinska J, Mégy C, Quiles M, Cazevieille C, Korchagina J, Bonnet-Wersinger D, Milea D, Hamel C, Pinton P, Thiry M, Lacampagne A, Delprat B, Delettre C (2018) ER-mitochondria cross-talk is regulated by the Ca2+ sensor NCS1 and is impaired in Wolfram syndrome. Sci Signal 11:eaaq1380. | Human | Fibroblast |
Isohanni 2018 Neurogenetics | 2018 | Isohanni P, Carroll CJ, Jackson CB, Pohjanpelto M, Lönnqvist T, Suomalainen A (2018) Defective mitochondrial ATPase due to rare mtDNA m.8969G>A mutation-causing lactic acidosis, intellectual disability, and poor growth. Neurogenetics 19:49-53. | Human | Skeletal muscle Blood cells Fibroblast |
Alston 2018 Am J Hum Genet | 2018 | Alston CL, Heidler J, Dibley MG, Kremer LS, Taylor LS, Fratter C, French CE, Glasgow RIC, Feichtinger RG, Delon I, Pagnamenta AT, Dolling H, Lemonde H, Aiton N, Bjørnstad A, Henneke L, Gärtner J, Thiele H, Tauchmannova K, Quaghebeur G, Houstek J, Sperl W, Raymond FL, Prokisch H, Mayr JA, McFarland R, Poulton J, Ryan MT, Wittig I, Henneke M, Taylor RW (2018) Bi-allelic mutations in NDUFA6 establish its role in early-onset isolated mitochondrial complex I deficiency. Am J Hum Genet 103:592-601. | Human | Fibroblast |
Einer 2018 Cell Mol Gastroenterol Hepatol | 2018 | Einer C, Leitzinger C, Lichtmannegger J, Eberhagen C, Rieder T, Borchard S, Wimmer R, Denk G, Popper B, Neff F, Polishchuk EV, Polishchuk RS, Hauck SM, von Toerne C, Müller JC, Karst U, Baral BS, DiSpirito AA, Kremer AE, Semrau J, Weiss KH, Hohenester S, Zischka H (2018) A high calorie diet aggravates mitochondrial dysfunction and triggers severe liver damage in Wilson disease rats. Cell Mol Gastroenterol Hepatol 7:571-96. | Rat | Liver |
Arnoux 2018 Elife | 2018 | Arnoux I, Willam M, Griesche N, Krummeich J, Watari H, Offermann N, Weber S, Narayan Dey P, Chen C, Monteiro O, Buettner S, Meyer K, Bano D, Radyushkin K, Langston R, Lambert JJ, Wanker E, Methner A, Krauss S, Schweiger S, Stroh A (2018) Metformin reverses early cortical network dysfunction and behavior changes in Huntington's disease. Elife 7:e38744. | Mouse | Nervous system |
Collard 2018 Mol Cell Biol | 2018 | Collard R, Majtan T, Park I, Kraus JP (2018) Import of TAT-conjugated propionyl coenzyme A carboxylase using models of propionic acidemia. Mol Cell Biol 38. pii: e00491-17. | Mouse | Liver |
Becker 2018 EMBO Rep | 2018 | Becker C, Kukat A, Szczepanowska K, Hermans S, Senft K, Brandscheid CP, Maiti P, Trifunovic A (2018) CLPP deficiency protects against metabolic syndrome but hinders adaptive thermogenesis. EMBO Rep 19. pii: e45126. | Mouse | Liver Fat |
Eimre 2018 Oxid Med Cell Longev | 2018 | Eimre M, Paju K, Peet N, Kadaja L, Tarrend M, Kasvandik S, Seppet J, Ivask M, Orlova E, Kõks S (2018) Increased mitochondrial protein levels and bioenergetics in the musculus rectus femoris of Wfs1-deficient mice. Oxid Med Cell Longev 2018:3175313. | Mouse | Skeletal muscle |
Ruiz 2017 Brain Pathol | 2017 | Ruiz M, Bégou M, Launay N, Ranea-Robles P, Bianchi P, López-Erauskin J, Morató L, Guilera C, Petit B, Vaurs-Barriere C, Guéret-Gonthier C, Bonnet-Dupeyron MN, Fourcade S, Auwerx J, Boespflug-Tanguy O, Pujol A (2017) Oxidative stress and mitochondrial dynamics malfunction are linked in Pelizaeus-Merzbacher disease. Brain Pathol 28:611-30. | Human Mouse | Nervous system Fibroblast |
Kang 2017 Mol Cell | 2017 | Kang Y, Stroud DA, Baker MJ, De Souza DP, Frazier AE, Liem M, Tull D, Mathivanan S, McConville MJ, Thorburn DR, Ryan MT, Stojanovski D (2017) Sengers Syndrome-associated mitochondrial acylglycerol kinase is a subunit of the human TIM22 protein import complex. Mol Cell 67:457-70. | Human | |
Chin 2017 US Patent | 2017 | Chin MT, Chien WM, Dinca A (2017) Methods for treating and preventing cardiomyopathy. US Patent WO2016094791A1. | Mouse | Heart |
Kennedy 2017 Skelet Muscle | 2017 | Kennedy TL, Moir L, Hemming S, Edwards B, Squire S, Davies K, Guiraud S (2017) Utrophin influences mitochondrial pathology and oxidative stress in dystrophic muscle. Skelet Muscle 7:22. | Mouse | Skeletal muscle |
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Baron 2010 Thesis | 2010 | Baron M (2010) Copy number variations of the mitochondrial DNA as potential cause of mitochondrial diseases. Thesis Rheinischen Friedrich-Wilhelms-Universitaet Bonn, Mathematisch-Naturwissenschaftlichen Fakultät, 110pp. | Human | Endothelial;epithelial;mesothelial cell Fibroblast |
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Phielix 2010 Diabetologia | 2010 | Phielix E, Meex R, Moonen-Kornips E, Hesselink MK, Schrauwen P (2010) Exercise training increases mitochondrial content and ex vivo mitochondrial function similarly in patients with type 2 diabetes and in control individuals. Diabetologia 53:1714-21. | Human | Skeletal muscle |
Martin 2010 Proc Natl Acad Sci U S A | 2010 | Martin B, Ji S, Maudsley S, Mattson MP (2010) "Control" laboratory rodents are metabolically morbid: why it matters. Proc Natl Acad Sci U S A 107:6127-33. | Human Mouse Rat | Nervous system |
Casasnovas 2009 J Med Genet | 2010 | Casasnovas C, Banchs I, Cassereau J, Gueguen N, Chevrollier A, Martínez-Matos JA, Bonneau D, Volpini V (2010) Phenotypic spectrum of MFN2 mutations in the Spanish population. J Med Genet 47:249-56. | Human | Nervous system Fibroblast |
Gomez-Duran 2010 Hum Mol Genet | 2010 | Gómez-Durán A, Pacheu-Grau D, López-Gallardo E, Díez-Sánchez C, Montoya J, López-Pérez MJ, Ruiz-Pesini E (2010) Unmasking the causes of multifactorial disorders: OXPHOS differences between mitochondrial haplogroups. Hum Mol Genet 19:3343-53. | Human | |
Chowdhury 2010 Diabetes | 2010 | Chowdhury SK, Zherebitskaya E, Smith DR, Akude E, Chattopadhyay S, Jolivalt CG, Calcutt NA, Fernyhough P (2010) Mitochondrial respiratory chain dysfunction in dorsal root ganglia of streptozotocin-induced diabetic rats and its correction by insulin treatment. Diabetes 59:1082-91. | Rat | Nervous system |
Cassereau 2009 Neurogenetics | 2009 | Cassereau J, Chevrollier A, Gueguen N, Malinge MC, Letournel F, Nicolas G, Richard L, Ferre M, Verny C, Dubas F, Procaccio V, Amati-Bonneau P, Bonneau D, Reynier P (2009) Mitochondrial Complex I deficiency in GDAP1-related autosomal dominant Charcot-Marie-Tooth disease (CMT2K). Neurogenetics 10:145-50. | Human | Nervous system |
Nochez 2009 Mol Vis | 2009 | Nochez Y, Arsene S, Gueguen N, Chevrollier A, Ferré M, Guillet V, Desquiret V, Toutain A, Bonneau D, Procaccio V, Amati-Bonneau P, Pisella PJ, Reynier P (2009) Acute and late-onset optic atrophy due to a novel OPA1 mutation leading to a mitochondrial coupling defect. Mol Vis 15:598-608. | Human | Fibroblast |
Iyer 2009 Mitochondrion | 2009 | Iyer S, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP (2009) Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression. Mitochondrion 9:196-203. | Human | Skeletal muscle Nervous system |
Lim 2009 PlosOne | 2009 | Lim S, Ahn SY, Song IC, Chung MH, Jang HC, Park KS, Lee KU, Pak YK, Lee HK (2009) Chronic exposure to the herbicide, atrazine, causes mitochondrial dysfunction and insulin resistance. PLoS One 4:e5186. | Rat | Skeletal muscle Liver |
Keeney 2009 Exp Neurol | 2009 | Keeney PM, Dunham LD, Quigley CK, Morton SL, Bergquist KE, Bennett JP (2009) Cybrid models of Parkinson's disease show variable mitochondrial biogenesis and genotype-respiration relationships. Exp Neurol 220:374-82. | Human | Blood cells Platelet |
Mantena 2009 Biochem J | 2009 | Mantena SK, Vaughn Jr DP, Andringa KK, Eccleston HB, King AL, Abrams GA, Doeller JE, Kraus DW, Darley-Usmar V, Bailey SM (2009) High fat diet induces dysregulation of hepatic oxygen gradients and mitochondrial function in vivo. Biochem J 417:183–93. | Mouse | Liver |
Jones 2009 Exp Neurol | 2009 | Jones TT, Brewer GJ (2009) Critical age-related loss of cofactors of neuron cytochrome c oxidase reversed by estrogen. Exp Neurol 215:212-9. | Rat | Nervous system |
Raboel 2009 Diabetes Obes Metab | 2009 | Raboel R, Hojberg PM, Almdal T, Boushel RC, Haugaard SB, Madsbad S, Dela F (2009) Improved glycaemic control decreases inner mitochondrial membrane leak in type 2 diabetes. Diabetes Obes Metab 11:355-60. | Human | Skeletal muscle |
Anderson 2009 J Am Coll Cardiol | 2009 | Anderson EJ, Kypson AP, Rodriguez E, Anderson CA, Lehr EJ, Neufer PD (2009) Substrate-specific derangements in mitochondrial metabolism and redox balance in the atrium of the type 2 diabetic human heart. J Am Coll Cardiol 54:1891-8. | Human | Heart |
Jesse 2009 CNS Neurosci Ther | 2009 | Jesse S, Steinacker P, Lehnert S, Gillardon F, Hengerer B, Otto M (2009) Neurochemical approaches in the laboratory diagnosis of Parkinson and Parkinson dementia syndromes: a review. CNS Neurosci Ther 15:157-82. | ||
Moisoi 2009 Cell Death Differ | 2009 | Moisoi N, Klupsch K, Fedele V, East P, Sharma S, Renton A, Plun-Favreau H, Edwards RE, Teismann P, Esposti MD, Morrison AD, Wood NW, Downward J, Martins LM (2009) Mitochondrial dysfunction triggered by loss of HtrA2 results in the activation of a brain-specific transcriptional stress response. Cell Death Differ 16:449-64. | Mouse | Nervous system Liver |
Lancel 2009 J Pharmacol Exp Ther | 2009 | Lancel S, Hassoun SM, Favory R, Decoster B, Motterlini R, Neviere R (2009) Carbon monoxide rescues mice from lethal sepsis by supporting mitochondrial energetic metabolism and activating mitochondrial biogenesis. J Pharmacol 329:641-48. | Mouse | Heart |
Myhill 2009 Int J Clin Exp Med | 2009 | Myhill S, Booth NE, McLaren-Howard J (2009) Chronic fatigue syndrome and mitochondrial dysfunction. Int J Clin Exp Med 2:1-16. | ||
Monge 2008 Mol Cell Biochem | 2008 | Monge C, Beraud N, Kuznetsov AV, Rostovtseva T, Sackett D, Schlattner U, Vendelin M, Saks V (2008) Regulation of respiration in brain mitochondria and synaptosomes: restrictions of ADP diffusion in situ, roles of tubulin, and mitochondrial creatine kinase. Mol Cell Biochem 318:147-65. | Rat | Nervous system |
Gruno 2008 J Gastroenterol | 2008 | Gruno M, Peet N, Tein A, Salupere R, Sirotkina M, Valle J, Peetsalu A, Seppet EK (2008) Atrophic gastritis: deficient complex I of the respiratory chain in the mitochondria of corpus mucosal cells. J Gastroenterol 43:780-8. | Human | Endothelial;epithelial;mesothelial cell |
Hansson 2008 Free Radic Biol Med | 2008 | Hansson Magnus J, Månsson R, Morota S, Uchino H, Kallur T, Sumi T, Ishii N, Shimazu M, Keep MF, Jegorov A, Elmér E (2008) Calcium-induced generation of reactive oxygen species in brain mitochondria is mediated by permeability transition. Free Radic Biol Med 45:284-94. | Rat | Nervous system Liver |
Rauchova 2008 J Bioenerg Biomembr | 2008 | Rauchova H, Drahota Z, Bergamini C, Fato R, Lenaz G (2008) Modification of respiratory-chain enzyme activities in brown adipose tissue mitochondria by idebenone (hydroxydecyl-ubiquinone). J Bioenerg Biomembr 40:85-93. | ||
Hein 2008 Hum Mol Genet | 2008 | Hein S, Schönfeld P, Kahlert S, Reiser G (2008) Toxic effects of X-linked adrenoleukodystrophy-associated, very long chain fatty acids on glial cells and neurons from rat hippocampus in culture. Hum Mol Genet 17:1750-61. | Rat | Nervous system |
Iyer 2008 Nature Precedings | 2008 | Iyer S, Thomas R, Portell F, Dunham L, Quigley C, Bennett JP (2008) Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression in G11778A leber’s hereditary optic neuropathy cybrid cells. Nature Precedings: hdl:10101/npre.2008.2084.1 | Nervous system | |
Juellig 2008 Proteomics | 2008 | Jüllig M, Hickey AJ, Chai CC, Skea GL, Middleditch MJ, Costa S, Choong SY, Philips AR, Cooper GJ (2008) Is the failing heart out of fuel or a worn engine running rich? A study of mitochondria in old spontaneously hypertensive rats. Proteomics 8:2556-72. | Rat | Heart |
Haas 2008 Mol Genet Metab | 2008 | Haas RH, Parikh S, Falk MJ, Saneto RP, Wolf NI, Darin N, Wong LJ, Cohen BH, Naviaux RK (2008) The in-depth evaluation of suspected mitochondrial disease. Mol Genet Metab 94:16-37. | Nervous system | |
Kuzelova 2008 Gen Physiol Biophys | 2008 | Kuzelova M, Adameova A, Sumbalova Z, Paulikova I, Harcarová A, Svec P, Kucharska J. (2008) The effect of simvastatin on coenzyme Q and antioxidant/oxidant balance in diabetic-hypercholesterolaemic rats. Gen Physiol Biophys. 27:291-8. | Rat | Heart Liver |
Deschauer 2006 Mol Genet Metab | 2006 | Deschauer M, Gizatullina Z, Schulze A, Pritsch M, Knöppel C, Knape M, Zierz S, Gellerich FN (2006) Molecular and biochemical investigations in fumarase deficiency. Mol Genet Metab 88:146-52. | Human | Fibroblast |
Kasparova 2006 Neurochem Int | 2006 | Kasparová S, Sumbalová Z, Bystrický P, Kucharská J, Liptaj T, Mlynárik V, Gvozdjáková A (2006) Effect of coenzyme Q10 and vitamin E on brain energy metabolism in the animal model of Huntington's disease. Neurochem Int 48:93-9. | Rat | Nervous system |
Larche 2006 J Am Coll Cardiol | 2006 | Larche J, Lancel S, Hassoun SM, Favory R, Decoster B, Marchetti P, Chopin C, Neviere R (2006) Inhibition of mitochondrial permeability transition prevents sepsis-induced myocardial dysfunction and mortality. J Am Coll Cardiol 48:377-85. | Human Mouse | Heart |
Raboel 2006 Appl Physiol Nutr Metab | 2006 | Raboel R, Boushel RC, Dela F (2006) Mitochondrial oxidative function and type 2 diabetes. Appl Physiol Nutr Metab 31:675-83. | Human | Skeletal muscle |
Blakely 2006 Pediatr Res | 2006 | Blakely EL, Rennie KJ, Jones L, Elstner M, Chrzanowska-Lightowlers ZMA, White CB, Shield JPH, Pilz DT, Turnbull DM, Poulton J, Taylor RW (2006) Sporadic intragenic inversion of the mitochondrial DNA MTND1 gene causing fatal infantile lactic acidosis. Pediatr Res 59:440-4. | Human | Fibroblast |
Honzik 2006 Placenta | 2006 | Honzik T, Drahota Z, Bohm M, Jesina P, Mracek T, Paul J, Zeman J, Houstek J (2006) Specific properties of heavy fraction of mitochondria from human-term placenta - Glycerophosphate-dependent hydrogen peroxide production. Placenta 27:348-56. | Human | |
Miller 2006 FEBS Lett | 2006 | Miller I, Gemeiner M, Gesslbauer B, Kungl A, Piskernik C, Haindl S, Nürnberger S, Bahrami Sl, Redl H, Kozlov AV (2006) Proteome analysis of rat liver mitochondria reveals a possible compensatory response to endotoxic shock. FEBS Lett 580:1257-62. | Rat | Liver |
Winkler-Stuck 2005 J Neural Transm | 2005 | Winkler-Stuck K, Kirches E, Mawrin C, Dietzmann K, Lins H, Wallesch CW, Kunz WS, Wiedemann FR (2005) Re-evaluation of the dysfunction of mitochondrial respiratory chain in skeletal muscle of patients with Parkinson's disease. J Neural Transm 112:499-518. | Human | Skeletal muscle |
Oezen 2005 Hum Mol Genet | 2005 | Oezen I, Rossmanith W, Forss-Petter S, Kemp S, Voigtlander T, Moser-Thier K, Wanders RJA, Bittner RE, Berger J (2005) Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency. Hum Mol Genet 14:1127-37. | Human Mouse | Skeletal muscle Nervous system Fibroblast |
Pecina 2004 Am J Physiol Cell Physiol | 2004 | Pecina P, Gnaiger E, Zeman J, Pronicka E, Houstek J (2004) Decreased affinity to oxygen of cytochrome c oxidase in Leigh syndrome caused by SURF1 mutations. Am J Physiol Cell Physiol 287:C1384-8. | Human | Fibroblast |
Pecina 2004 Physiol Res | 2004 | Pecina P, Houstkova H, Hansikova H, Zeman J, Houstek J (2004) Genetic defects of cytochrome c oxidase assembly. Physiol Res 53 Suppl 1:S213-23. | Human | Skeletal muscle Fibroblast |
Gellerich 2004 Mol Cell Biochem | 2004 | Gellerich FN, Trumbeckaite S, Müller T, Deschauer M, Chen Y, Gizatullina Z, Zierz S (2004) Energetic depression caused by mitochondrial dysfunction. Mol Cell Biochem 256/257:391-405. | ||
Winkler-Stuck 2004 J Neurol Sci | 2004 | Winkler-Stuck K, Wiedemann FR, Wallesch CW, Kunz WS (2004) Effect of coenzyme Q10 on the mitochondrial function of skin fibroblasts from Parkinson patients. J Neurol Sci 220:41-8. | Human | Fibroblast |
Jesina 2004 Biochem J | 2004 | Jesina P, Tesarova M, Fornuskova D, Vojtiskova A, Pecina P, Kaplanova V, Hansikova H, Zeman J, Houstek J (2004) Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206. Biochem J 383:561-71. | Human | Fibroblast |
Wenchich 2003 Physiol Res | 2003 | Wenchich L, Drahota Z, Honzík T, Hansíková H, Tesarová M, Zeman J, Houstek J (2003) Polarographic evaluation of mitochondrial enzymes activity in isolated mitochondria and in permeabilized human muscle cells with inherited mitochondrial defects. Physiol Res 52:781-88. | Human | Skeletal muscle |
Pecina 2003 Biochim Biophys Acta | 2003 | Pecina P, Capkova M, Chowdhury SK, Drahota Z, Dubot A, Vojtiskova A, Hansikova H, Houstekova H, Zeman J, Godinot C, Houstek J (2003) Functional alteration of cytochrome c oxidase by SURF1 mutations in Leigh syndrome. Biochim Biophys Acta 1639:53-63. | Human | Endothelial;epithelial;mesothelial cell Fibroblast |
Vielhaber 2003 Epilepsia | 2003 | Vielhaber S, Von Oertzen JH, Kudin AF, Schoenfeld A, Menzel C, Biersack HJ, Kral T, Elger CE, Kunz WS (2003) Correlation of hippocampal glucose oxidation capacity and interictal FDG-PET in temporal lobe epilepsy. Epilepsia 44:193-9. | Human | Nervous system |
Kudin 2002 Eur J Neurosci | 2002 | Kudin AP, Kudina TA, Seyfried J, Vielhaber S, Beck H, Elger CE, Kunz WS (2002) Seizure-dependent modulation of mitochondrial oxidative phosphorylation in rat hippocampus. Eur J Neurosci 15:1105-14. | Rat | Nervous system |
Varlamov 2002 Hum Mol Genet | 2002 | Varlamov DA, Kudin AP, Vielhaber S, Schroder R, Sassen R, Becker A, Kunz D, Haug K, Rebstock J, Heils A, Elger CE, Kunz WS (2002) Metabolic consequences of a novel missense mutation of the mtDNA CO I gene. Hum Mol Genet 11:1797-805. | Human | Skeletal muscle |
Braun 2001 Biochim Biophys Acta | 2001 | Braun U, Paju K, Eimre M, Seppet E, Orlova E, Kadaja L, Trumbeckaite S, Gellerich FN, Zierz S, Jockusch H, Seppet EK (2001) Lack of dystrophin is associated with altered integration of the mitochondria and ATPases in slow-twitch muscle cells of MDX mice. Biochim Biophys Acta 1505:258-70. | Mouse | Heart Skeletal muscle |
Vielhaber 2000 Biochem Soc Trans | 2000 | Vielhaber S, Kudin A, Schroder R, Elger CE, Kunz WS (2000) Muscle fibres: applications for the study of the metabolic consequences of enzyme deficiencies in skeletal muscle. Biochem Soc Trans 28:159-64. | Human | Skeletal muscle |
Kunz 2000 Ann Neurol | 2000 | Kunz WS, Kudin AP, Vielhaber S, Blumecke I, Zuschratter W, Schramm J, Beck H, Elger CE (2000) Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy. Ann Neurol 48:766-73. | Human | Nervous system |
Kemp 2000 Thermochim Acta (Vol 355) | 2000 | Kemp RB (2000) "Fire burn and cauldron bubble" (W. Shakespeare): what the calorimetric-respirometric (CR) ratio does for our understanding of cells? Thermochim Acta 355:115-24. | ||
Oexle 1999 Biochim Biophys Acta | 1999 | Oexle H, Gnaiger E, Weiss G (1999) Iron-dependent changes in cellular energy metabolism: influence on citric acid cycle and oxidative phosphorylation. Biochim Biophys Acta 1413:99-107. | Human | Blood cells |
Vielhaber 1999 J Neurol Sci | 1999 | Vielhaber S, Winkler K, Kirches E, Kunz D, Büchner M, Feistner H, Elger CE, Ludolph AC, Riepe MW, Kunz WS (1999) Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis. J Neurol Sci 169:133-9. | Human | Skeletal muscle Nervous system |
Wiedemann 1998 J Neurol Sci | 1998 | Wiedemann FR, Winkler K, Kuznetsov AV, Bartels C, Vielhaber S, Feistner H, Kunz WS (1998) Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis. J Neurol Sci 156:65-72. | Human | Skeletal muscle |
Kirches 1998 J Inherit Metab Dis | 1998 | Kirches EJF, Winkler K, Warich-Kirches M, Szibor R, Wien F, Kunz WS, von Bossanyi P, Bajaj PK, Dietzmann K (1998) mtDNA depletion and impairment of mitochondrial function in a case of a multisystem disorder including severe myopathy. J Inherit Metab Dis 21:400-8. | Human | Skeletal muscle |
Halangk 1997 Zentralbl Chir | 1997 | Halangk W, Matthias R, Nedelev B, Schild L, Meyer F, Schulz H-U, Lippert H (1997) Beeinträchtigung der Energiebereitstellung von Pankreasmitochondrien bei experimenteller akuter Pankreatitis. Zentralbl Chir 122:305-8. | ||
Kuznetsov 1997 Biochim Biophys Acta | 1997 | Kuznetsov AV, Winkler K, Kirches E, Lins H, Feistner H, Kunz WS (1997) Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases. Biochim Biophys Acta 1360:142-50. | Skeletal muscle | |
Kunz 1997 Anal Biochem | 1997 | Kunz D, Luley C, Winkler K, Lins H, Kunz WS (1997) Flow cytometric detection of mitochondrial dysfunction in subpopulations of human mononuclear cells. Anal Biochem 246:218-24. | Blood cells Lymphocyte | |
Kunz 1997 Mol Cell Biochem | 1997 | Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW (1997) Detection of mitochondrial defects by laser fluorimetry. Mol Cell Biochem 174:97-100. | Human | Skeletal muscle |
Kirches 1996 Saponin | 1996 | Kirches E, Winkler K, Kuznetsov AV, Lins H, von Bossanyi P, Dietzmann K, Frank B, Feistner H, Kunz WS (1996) Laserfluoreszenz-Studien der Mitochondrienfunktion in Saponin-permeabilisierten Skelettmuskelfasern von Patienten mit chronisch progressiver externer Ophthalmoplegie. Jahrb Neuromusk Erkrankungen 1996, Arcis Verlag:166-9. | Human | Skeletal muscle |
Winkler 1995 Biochim Biophys Acta | 1995 | Winkler K, Kuznetsov AV, Lins H, Kirches E, von Bossanyi P, Dietzmann K, Frank B, Feistner H, Kunz WS (1995) Laser-excited fluorescence studies of mitochondrial function in saponin-skinned skeletal muscle fibers of patients with chronic progressive external ophthalmoplegia. Biochim Biophys Acta 1272:181-4. | Human | Skeletal muscle |
Kunz 1995 Biochem Mol Med | 1995 | Kunz D, Luley C, Fritz S, Bohnensack R, Winkler K, Kunz WS, Wallesch CW (1995) Oxygraphic evaluation of mitochondrial function in digitonin-permeabilized mononuclear cells and cultured skin fibroblasts of patients with chronic progressive external ophthalmoplegia. Biochem Mol Med 54:105-11. | Human | |
Sperl 1994 J Inher Metab Dis | 1994 | Sperl W, Skladal D, Lanznaster N, Schranzhofer R, Zaunschirm G, Gnaiger E, Gellerich FN (1994) Polarographic studies of saponin-skinned muscle fibers in patients with mitochondrial myopathies. J Inher Metab Dis 17:307-10. | Human | Skeletal muscle |
Kuznetsov 1994 BTK-275 | 1994 | Kuznetsov AV, Clark JF, Winkler K, Kunz WS (1994) Change in flux control coefficient of cytochrome c oxidase in copper deficient mottled brindled mice. In: What is Controlling Life? (Gnaiger E, Gellerich FN, Wyss M, eds) Modern Trends in BioThermoKinetics 3. Innsbruck Univ Press:275-77. |
O2k-Publications: Mitochondrial Disease; Degenerative Disease and Defect - Abstracts
Sort in ascending/descending order by a click on one of the small symbols in squares below.
Year | Reference | Organism | Tissue;cell | |
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Xia 2024 Nat Metab | Xia W, Veeragandham P, Cao Y, Yayun Xu Y, Rhyne TE, Qian J, Hung C-W, Zhao P, Jones Y, Gao H, Liddle C, Yu RT, Downes M, Evans RM, Rydén M, Wabitsch M, Wang Z, Hakozaki H, Schöneberg J, Reilly SM, Huang J, Saltiel AR (2024) Obesity causes mitochondrial fragmentation and dysfunction in white adipocytes due to RalA activation. Nat Metab 6:273–89. https://doi.org/10.1038/s42255-024-00978-0 | Human Mouse | Fat | |
Pegoraro 2024 Adv Drug Deliv Rev | Pegoraro C, Domingo I, Conejos-Sánchez I, Vicent MJ (2024) Unlocking the mitochondria for nanomedicine-based treatments: overcoming biological barriers, improving designs, and selecting verification techniques. | |||
Meldau 2024 Mol Genet Metab Rep | Meldau S, Ackermann S, Riordan G, van der Watt GF, Spencer C, Raga S, Khan K, Blackhurst DM, van der Westhuizen FH (2024) A novel mitochondrial DNA variant in MT-ND6: m.14430A>C p.(Trp82Gly) identified in a patient with Leigh syndrome and complex I deficiency. Mol Genet Metab Rep 39:101078. https://doi.org/10.1016/j.ymgmr.2024.101078 | Human | Fibroblast | |
Schober 2022 Hum Mol Genet | Schober FA, Tang JX, Sergeant K, Moedas MF, Zierz CM, Moore D, Smith C, Lewis D, Guha N, Hopton S, Falkous G, Lam A, Pyle A, Poulton J, Gorman GS, Taylor RW, Freyer C, Wredenberg A (2022) Pathogenic SLC25A26 variants impair SAH transport activity causing mitochondrial disease. https://doi.org/10.1093/hmg/ddac002 | Mouse Drosophila | Fibroblast | |
Spielmann 2022 Mamm Genome | Spielmann N, Schenkl C, Komlódi T, da Silva-Buttkus P, Heyne E, Rohde J, Amarie OV, Rathkolb B, Gnaiger E, Doenst T, Fuchs H, Gailus-Durner V, de Angelis MH, Szibor M (2022) Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction. Mamm Genome 10.1007/s00335-022-09973-w | Mouse | Heart | |
Munoz-Pujol 2022 Brain Pathol | Muñoz-Pujol G, Ortigoza-Escobar JD, Paredes-Fuentes AJ, Jou C, Ugarteburu O, Gort L, Yubero D, García-Cazorla A, O'Callaghan M, Campistol J, Muchart J, Yépez VA, Gusic M, Gagneur J, Prokisch H, Artuch R, Ribes A, Urreizti R, Tort F (2022) Leigh syndrome is the main clinical characteristic of PTCD3 deficiency. https://doi.org/10.1111/bpa.13134 | Human | Fibroblast | |
Lee 2022 Hum Mol Genet | Lee RG, Balasubramaniam S, Stentenbach M, Kralj T, McCubbin T, Padman B, Smith J, Riley LG, Priyadarshi A, Peng L, Nuske MR, Webster R, Peacock K, Roberts P, Stark Z, Lemire G, Ito YA; Care4Rare Canada Consortium, Boycott KM, Geraghty MT, van Klinken JB, Ferdinandusse S, Zhou Y, Walsh R, Marcellin E, Thorburn DR, Rosciolli T, Fletcher J, Rackham O, Vaz FM, Reid GE, Filipovska A (2022) Deleterious variants in CRLS1 lead to cardiolipin deficiency and cause an autosomal recessive multi-system mitochondrial disease. https://doi.org/10.1093/hmg/ddac040 | Human | Fibroblast | |
Aasander Frostner 2022 MitoFit | Åsander Frostner E, Simón Serrano S, Chamkha I, Donnelly E, Elmér E, Hansson MJ (2022) Towards a treatment for mitochondrial disease: current compounds in clinical development. https://doi.org/10.26124/mitofit:2022-0014 — 2022-06-28 published in Bioenerg Commun 2022.4. | Human | ||
Zhao 2022 J Cell Sci | Zhao Q, Yan S, Lu J, Parker DJ, Wu H, Sun Q, Crossman DK, Liu S, Wang Q, Sesaki H, Mitra K, Liu K, Jiao K (2022) Drp1 regulates transcription of ribosomal protein genes in embryonic hearts. https://doi.org/10.1242/jcs.258956 | Mouse | Heart | |
Aasander Frostner 2022 BEC | Åsander Frostner E, Simón Serrano S, Chamkha I, Donnelly E, Elmér E, Hansson MJ (2022) Towards a treatment for mitochondrial disease: current compounds in clinical development. Bioenerg Commun 2022.4. https://doi.org/10.26124/bec:2022-0004 | Human | ||
Zhou 2022 J Hum Genet | Zhou X, Lou X, Zhou Y, Xie Y, Han X, Dong Q, Ying X, Laurentinah MR, Zhang L, Chen Z, Li D, Fang H, Lyu J, Yang Y, Wang Y (2022) Novel biallelic mutations in TMEM126B cause splicing defects and lead to Leigh-like syndrome with severe complex I deficiency. https://doi.org/10.1038/s10038-022-01102-4 | Human | Lymphocyte | |
Adant 2022 Mol Metab | Adant I, Bird M, Decru B, Windmolders P, Wallays M, de Witte P, Rymen D, Witters P, Vermeersch P, Cassiman D, Ghesquière B (2022) Pyruvate and uridine rescue the metabolic profile of OXPHOS dysfunction. https://doi.org/10.1016/j.molmet.2022.101537 | Human | Fibroblast | |
Belal 2022 Biomedicines | Belal S, Goudenège D, Bocca C, Dumont F, Chao De La Barca JM, Desquiret-Dumas V, Gueguen N, Geffroy G, Benyahia R, Kane S, Khiati S, Bris C, Aranyi T, Stockholm D, Inisan A, Renaud A, Barth M, Simard G, Reynier P, Letournel F, Lenaers G, Bonneau D, Chevrollier A, Procaccio V (2022) Glutamate-induced deregulation of krebs cycle in mitochondrial encephalopathy lactic acidosis syndrome stroke-like episodes (MELAS) syndrome is alleviated by ketone body exposure. https://doi.org/10.3390/biomedicines10071665 | Human | Neuroblastoma | |
Inak 2021 Nat Commun | Inak G, Rybak-Wolf A, Lisowski P, Pentimalli TM, Jüttner R, Glažar P, Uppal K, Bottani E, Brunetti D, Secker C, Zink A, Meierhofer D, Henke MT, Dey M, Ciptasari U, Mlody B, Hahn T, Berruezo-Llacuna M, Karaiskos N, Di Virgilio M, Mayr JA, Wortmann SB, Priller J, Gotthardt M, Jones DP, Mayatepek E, Stenzel W, Diecke S, Kühn R, Wanker EE, Rajewsky N, Schuelke M, Prigione A (2021) Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome. Nat Commun 12:1929. | Nervous system | ||
Gueguen 2021 Methods Mol Biol | Gueguen N, Lenaers G, Reynier P, Weissig V, Edeas M (2021) Mitochondrial dysfunction in mitochondrial medicine: current limitations, pitfalls, and tomorrow. https://doi.org/10.1007/978-1-0716-1266-8_1 | |||
Friederich 2021 Mol Genet Metab | Friederich MW, Geddes GC, Wortmann SB, Punnoose A, Wartchow E, Knight KM, Prokisch H, Creadon-Swindell G, Mayr JA, Van Hove JLK (2021) Pathogenic variants in MRPL44 cause infantile cardiomyopathy due to a mitochondrial translation defect. Mol Genet Metab 133:362-71. | Human | Fibroblast | |
Cretin 2021 EMBO Mol Med | Cretin E, Lopes P, Vimont E, Tatsuta T, Langer T, Gazi A, Sachse M, Yu-Wai-Man P, Reynier P, Wai T (2021) High-throughput screening identifies suppressors of mitochondrial fragmentation in OPA1 fibroblasts. EMBO Mol Med 13:13579. | Mouse | Fibroblast | |
Sun 2021 J Genet Genomics | Sun Y, Wei X, Fang F, Shen Y, Wei H, Li J, Ye X, Zhan Y, Ye X, Liu X, Yang W, Li Y, Geng X, Huang X, Ruan Y, Qin Z, Yi S, Lyu J, Fang H, Yu Y (2021) HPDL deficiency causes a neuromuscular disease by impairing the mitochondrial respiration. J Genet Genomics 48:727-36. | Human | HeLa Lymphocyte | |
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