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Primary skin fibroblasts as a model of Parkinson's disease.
Auburger G, Klinkenberg M, Drost J, Marcus K, Morales-Gordo B, Kunz WS, Brandt U, Broccoli V, Reichmann H, Gispert S, Jendrach M. Auburger G, et al. Among authors: kunz ws. Mol Neurobiol. 2012 Aug;46(1):20-7. doi: 10.1007/s12035-012-8245-1. Epub 2012 Feb 19. Mol Neurobiol. 2012. PMID: 22350618 Free PMC article. Review.
Parkinson phenotype in aged PINK1-deficient mice is accompanied by progressive mitochondrial dysfunction in absence of neurodegeneration.
Gispert S, Ricciardi F, Kurz A, Azizov M, Hoepken HH, Becker D, Voos W, Leuner K, Müller WE, Kudin AP, Kunz WS, Zimmermann A, Roeper J, Wenzel D, Jendrach M, García-Arencíbia M, Fernández-Ruiz J, Huber L, Rohrer H, Barrera M, Reichert AS, Rüb U, Chen A, Nussbaum RL, Auburger G. Gispert S, et al. Among authors: kunz ws. PLoS One. 2009 Jun 3;4(6):e5777. doi: 10.1371/journal.pone.0005777. PLoS One. 2009. PMID: 19492057 Free PMC article.
Mutant desmin substantially perturbs mitochondrial morphology, function and maintenance in skeletal muscle tissue.
Winter L, Wittig I, Peeva V, Eggers B, Heidler J, Chevessier F, Kley RA, Barkovits K, Strecker V, Berwanger C, Herrmann H, Marcus K, Kornblum C, Kunz WS, Schröder R, Clemen CS. Winter L, et al. Among authors: kunz ws. Acta Neuropathol. 2016 Sep;132(3):453-73. doi: 10.1007/s00401-016-1592-7. Epub 2016 Jul 8. Acta Neuropathol. 2016. PMID: 27393313 Free PMC article.
New insights into the metabolic consequences of large-scale mtDNA deletions: a quantitative analysis of biochemical, morphological, and genetic findings in human skeletal muscle.
Schröder R, Vielhaber S, Wiedemann FR, Kornblum C, Papassotiropoulos A, Broich P, Zierz S, Elger CE, Reichmann H, Seibel P, Klockgether T, Kunz WS. Schröder R, et al. Among authors: kunz ws. J Neuropathol Exp Neurol. 2000 May;59(5):353-60. doi: 10.1093/jnen/59.5.353. J Neuropathol Exp Neurol. 2000. PMID: 10888364
Impairment of mitochondrial oxidative phosphorylation in skin fibroblasts of SALS and FALS patients is rescued by in vitro treatment with ROS scavengers.
Debska-Vielhaber G, Miller I, Peeva V, Zuschratter W, Walczak J, Schreiber S, Petri S, Machts J, Vogt S, Szczepanowska J, Gellerich FN, Hermann A, Vielhaber S, Kunz WS. Debska-Vielhaber G, et al. Among authors: kunz ws. Exp Neurol. 2021 May;339:113620. doi: 10.1016/j.expneurol.2021.113620. Epub 2021 Jan 23. Exp Neurol. 2021. PMID: 33497646
Epileptic focus and alteration of metabolism.
Otáhal J, Folbergrová J, Kovacs R, Kunz WS, Maggio N. Otáhal J, et al. Among authors: kunz ws. Int Rev Neurobiol. 2014;114:209-43. doi: 10.1016/B978-0-12-418693-4.00009-1. Int Rev Neurobiol. 2014. PMID: 25078504 Review.
Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease.
Kornblum C, Nicholls TJ, Haack TB, Schöler S, Peeva V, Danhauser K, Hallmann K, Zsurka G, Rorbach J, Iuso A, Wieland T, Sciacco M, Ronchi D, Comi GP, Moggio M, Quinzii CM, DiMauro S, Calvo SE, Mootha VK, Klopstock T, Strom TM, Meitinger T, Minczuk M, Kunz WS, Prokisch H. Kornblum C, et al. Among authors: kunz ws. Nat Genet. 2013 Feb;45(2):214-9. doi: 10.1038/ng.2501. Epub 2013 Jan 13. Nat Genet. 2013. PMID: 23313956 Free PMC article.
Mitofusin 2 mutations affect mitochondrial function by mitochondrial DNA depletion.
Vielhaber S, Debska-Vielhaber G, Peeva V, Schoeler S, Kudin AP, Minin I, Schreiber S, Dengler R, Kollewe K, Zuschratter W, Kornblum C, Zsurka G, Kunz WS. Vielhaber S, et al. Among authors: kunz ws. Acta Neuropathol. 2013 Feb;125(2):245-56. doi: 10.1007/s00401-012-1036-y. Epub 2012 Aug 28. Acta Neuropathol. 2013. PMID: 22926664
295 results