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Wolfram Kunz

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Henriette J Tschampa; Horst Urbach; Susanne Greschus; Wolfram S Kunz; Cornelia Kornblum
Neuroimaging characteristics in mitochondrial encephalopathies associated with the m.3243A>G MTTL1 mutation.
Stefanie Schreiber; Claus Tempelmann; Reinhard Dengler; Helmut Feistner; Michael Goertler; Sibylle Jakubiczka; Katja Kollewe; Cornelia Kornblum; Siegfried Kropf; Wolfram S Kunz; Christian Mawrin; Andreas Oldag; Cordula Scherlach; Ariel Schoenfeld; Frank Schreiber; Stefan Vielhaber
Sonography of the median nerve in CMT1A, CMT2A, CMTX, and HNPP.
Gábor Zsurka; Wolfram S Kunz
Mitochondrial involvement in neurodegenerative diseases.

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All Publications

2013: Henriette J Tschampa; Horst Urbach; Susanne Greschus; Wolfram S Kunz; Cornelia Kornblum
Neuroimaging characteristics in mitochondrial encephalopathies associated with the m.3243A>G MTTL1 mutation.
Journal of neurology 2013;260(4):1071-80.
2013: Stefanie Schreiber; Claus Tempelmann; Reinhard Dengler; Helmut Feistner; Michael Goertler; Sibylle Jakubiczka; Katja Kollewe; Cornelia Kornblum; Siegfried Kropf; Wolfram S Kunz; Christian Mawrin; Andreas Oldag; Cordula Scherlach; Ariel Schoenfeld; Frank Schreiber; Stefan Vielhaber
Sonography of the median nerve in CMT1A, CMT2A, CMTX, and HNPP.
Muscle & nerve 2013;47(3):385-95.
2013: Gábor Zsurka; Wolfram S Kunz
Mitochondrial involvement in neurodegenerative diseases.
IUBMB life 2013;65(3):263-72.
2013: Dennis Lal; Holger Lerche; Costin Leu; Rikke Steensbjerre Møller; Bernd A Neubauer; Peter Nürnberg; Janina S Ried; Thomas Sander; Christoph J Schankin; Rainer Surges; Holger Trucks; Frank Visscher; Yvonne G Weber; Fritz Zimprich; Felicitas Becker; Carolien G F de Kovel; Andre Franke; Helle Hjalgrim; Thomas Illig; Bobby P C Koeleman; Wolfram S Kunz
Rare exonic deletions of the RBFOX1 gene increase risk of idiopathic generalized epilepsy.
Epilepsia 2013;54(2):265-71.
2013: Cornelia Kornblum; Wolfram S Kunz; Thomas Meitinger; Michal Minczuk; Maurizio Moggio; Vamsi K Mootha; Thomas J Nicholls; Viktoriya Peeva; Sarah E Calvo; Giacomo P Comi; Katharina Danhauser; Salvatore DiMauro; Tobias B Haack; Kerstin Hallmann; Arcangela Iuso; Thomas Klopstock; Catarina M Quinzii; Dario Ronchi; Joanna Rorbach; Susanne Schöler; Monica Sciacco; Tim M Strom; Thomas Wieland; Gabor Zsurka; Holger Prokisch
Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease.
Nature genetics 2013;45(2):214-9.
2013: Rikke Steensbjerre Møller; Hiltrud Muhle; Ina-Maria Rückert; Thomas Sander; Stefan Schreiber; Claudia Schurmann; Ulrich Stephani; Niels Tommerup; Holger Trucks; Yvonne G Weber; H-Erich Wichmann; Peter Wolf; Dieter Dennig; Jan P Ernst; Andre Franke; Hans J Grabe; Ingo Helbig; Helle Hjalgrim; Monika Kautza; Laura L Klitten; Wolfram S Kunz; Holger Lerche; Heather C Mefford
Exon-disrupting deletions of NRXN1 in idiopathic generalized epilepsy.
Epilepsia 2013;54(2):256-64.
2013: Stefan Vielhaber; Gábor Zsurka; Werner Zuschratter; Grazyna Debska-Vielhaber; Reinhard Dengler; Katja Kollewe; Cornelia Kornblum; Alexei P Kudin; Irina Minin; Viktoriya Peeva; S Schoeler; Stefanie Schreiber; Wolfram S Kunz
Mitofusin 2 mutations affect mitochondrial function by mitochondrial DNA depletion.
Acta neuropathologica 2013;125(2):245-56.
2013: Christoph Helmstaedter; Christian E Elger; Wolfram S Kunz; Yoan Mihov; Peter Nuernberg; Susanne Schoch; Rainer Surges; Holger Thiele; Mohammad R Toliat; René Hurlemann
Genetic variation in dopaminergic activity is associated with the risk for psychiatric side effects of levetiracetam.
Epilepsia 2013;54(1):36-44.
2012: Alexei P Kudin; Bartłomiej Augustynek; Richard Kovács; Anja Kerstin Lehmann; Wolfram S Kunz
The contribution of thioredoxin-2 reductase and glutathione peroxidase to H(2)O(2) detoxification of rat brain mitochondria.
Biochimica et biophysica acta 2012;1817(10):1901-6.
2012: Katja Jennissen; Stefanie Kipschull; Wolfram S Kunz; Michaela Liebig-Gonglach; Reinhard Fässler; Michaela-Rosemarie Hermann; Franziska Siegel; Sander van Dooren; Alexander Pfeifer
A VASP-Rac-soluble guanylyl cyclase pathway controls cGMP production in adipocytes.
Science signaling 2012;5(239):ra62.
2012: Georg Auburger; Ulrich Brandt; Vania Broccoli; Jessica Drost; Suzana Gispert; Michael Klinkenberg; Wolfram S Kunz; Katrin Marcus; Blas Morales-Gordo; Heinz Reichmann; Marina Jendrach
Primary skin fibroblasts as a model of Parkinson's disease.
Molecular neurobiology 2012;46(1):20-7.
2012: Benjamin Becker; Justin S Feinstein; Merve Aydin; Keith M Kendrick; Wolfram S Kunz; Wolfgang Maier; Andreas Matusch; Yoan Mihov; Ana-Maria Oros-Peusquens; Harald Reich; Dirk Scheele; Thomas Schlaepfer; Nadim J Shah; Horst Urbach; Karl Zilles; René Hurlemann
Fear processing and social networking in the absence of a functional amygdala.
Biological psychiatry 2012;72(1):70-7.
2012: Dominika Malinska; Malgorzata Bejtka; Alexei P Kudin; Wolfram S Kunz
Changes in mitochondrial reactive oxygen species synthesis during differentiation of skeletal muscle cells.
Mitochondrion 2012;12(1):144-8.
2012: Jaroslava Folbergrová; Wolfram S Kunz
Mitochondrial dysfunction in epilepsy.
Mitochondrion 2012;12(1):35-40.
2011: David-Marian Otte; Britta Sommersberg; Christoph W Turck; Oznur Yilmaz; Onder Albayram; Heinz Beck; Andras Bilkei-Gorzó; Eva Drews; Michaela D Filiou; Pamela Frisch; Catalina Guerrero; Alexei Kudin; Wolfram S Kunz; Andreas Zimmer
N-acetyl cysteine treatment rescues cognitive deficits induced by mitochondrial dysfunction in G72/G30 transgenic mice.
Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology 2011;36(11):2233-43.
2011: Joanna D Stewart; Susanne Schoeler; Kamil S Sitarz; Rita Horvath; Kerstin Hallmann; Angela Pyle; Patrick Yu-Wai-Man; Robert W Taylor; David Samuels; Wolfram S Kunz; Patrick F Chinnery
POLG mutations cause decreased mitochondrial DNA repopulation rates following induced depletion in human fibroblasts.
Biochimica et biophysica acta 2011;1812(3):321-5.
2011: Pitt Niehusmann; Rainer Surges; Randi D von Wrede; Christian E Elger; Jörg Wellmer; Jens Reimann; Horst Urbach; Stefan Vielhaber; Christian G Bien; Wolfram S Kunz
Mitochondrial dysfunction due to Leber's hereditary optic neuropathy as a cause of visual loss during assessment for epilepsy surgery.
Epilepsy & behavior : E&B 2011;20(1):38-43.
2010: Gábor Zsurka; Wolfram S Kunz
Mitochondrial dysfunction in neurological disorders with epileptic phenotypes.
Journal of bioenergetics and biomembranes 2010;42(6):443-8.
2010: Xinhong Guo; Konstantin Yu Popadin; Natalya Markuzon; Yuriy L Orlov; Yevgenya Kraytsberg; Kim J Krishnan; Gábor Zsurka; Douglas M Turnbull; Wolfram S Kunz; Konstantin Khrapko
Repeats, longevity and the sources of mtDNA deletions: evidence from 'deletional spectra'.
Trends in genetics : TIG 2010;26(8):340-3.
2010: Dominika Malinska; Bogusz Kulawiak; Alexei P Kudin; Richard Kovacs; Christine Huchzermeyer; Oliver Kann; Adam Szewczyk; Wolfram S Kunz
Complex III-dependent superoxide production of brain mitochondria contributes to seizure-related ROS formation.
Biochimica et biophysica acta 2010;1797(6-7):1163-70.
2010: Dominika Malinska; Sandra R Mirandola; Wolfram S Kunz
Mitochondrial potassium channels and reactive oxygen species.
FEBS letters 2010;584(10):2043-8.
2010: Gabor Zsurka; K G Hampel; I Nelson; C Jardel; S R Mirandola; Robert Sassen; Cornelia Kornblum; P Marcorelles; S Lavoué; A Lombès; Wolfram S Kunz
Severe epilepsy as the major symptom of new mutations in the mitochondrial tRNA(Phe) gene.
Neurology 2010;74(6):507-12.
2010: Gabor Zsurka; Tatiana A Kudina; Viktoriya Peeva; Kerstin Hallmann; Christian E Elger; Konstantin Khrapko; Wolfram S Kunz
Distinct patterns of mitochondrial genome diversity in bonobos (Pan paniscus) and humans.
BMC evolutionary biology 2010;10():270.
2010: Dominika Malinska; Bogusz Kulawiak; Antoni Wrzosek; Wolfram S Kunz; Adam Szewczyk
The cytoprotective action of the potassium channel opener BMS-191095 in C2C12 myoblasts is related to the modulation of calcium homeostasis.
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology 2010;26(2):235-46.
2009: G Debska-Vielhaber; M M Godlewski; Anna Kicinska; Jolanta Skalska; Bogusz Kulawiak; M Piwonska; Krzysztof Zablocki; Wolfram S Kunz; Adam Szewczyk; T Motyl
Large-conductance K+ channel openers induce death of human glioma cells.
Journal of physiology and pharmacology : an official journal of the Polish Physiological Society 2009;60(4):27-36.
2009: Katarzyna Choma; Piotr Bednarczyk; Izabela Koszela-Piotrowska; Bogusz Kulawiak; Alexei Kudin; Wolfram S Kunz; Krzysztof Dołowy; Adam Szewczyk
Single channel studies of the ATP-regulated potassium channel in brain mitochondria.
Journal of bioenergetics and biomembranes 2009;41(4):323-34.
2009: Alexei P Kudin; Gábor Zsurka; Christian E Elger; Wolfram S Kunz
Mitochondrial involvement in temporal lobe epilepsy.
Experimental neurology 2009;218(2):326-32.
2009: David M Otte; Andras Bilkei-Gorzó; Michaela D Filiou; Christoph W Turck; Oznur Yilmaz; Martin Ingo Holst; Karl Schilling; Rami Abou Jamra; Johannes Schumacher; Isabel Benzel; Wolfram S Kunz; Heinz Beck; Andreas Zimmer
Behavioral changes in G72/G30 transgenic mice.
European neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology 2009;19(5):339-48.
2009: M Boes; J Bauer; Horst Urbach; Christian E Elger; S Frank; M Baron; G Zsurka; Wolfram S Kunz; Cornelia Kornblum
Proof of progression over time: finally fulminant brain, muscle, and liver affection in Alpers syndrome associated with the A467T POLG1 mutation.
Seizure : the journal of the British Epilepsy Association 2009;18(3):232-4.
2009: Julia Gekeler; Gábor Zsurka; Wolfram S Kunz; Simon F Preuss; Jens Peter Klussmann; Orlando Guntinas-Lichius; Rudolf J Wiesner
Clonal expansion of different mtDNA variants without selective advantage in solid tumors.
Mutation research 2009;662(1-2):28-32.
2009: Jolanta Skalska; Piotr Bednarczyk; Marta Piwońska; Bogusz Kulawiak; Grzegorz Wilczynski; Krzysztof Dołowy; Alexei P Kudin; Wolfram S Kunz; Adam Szewczyk
Calcium ions regulate K(+) uptake into brain mitochondria: The evidence for a novel potassium channel.
International journal of molecular sciences 2009;10(3):1104-20.
2009: Adam Szewczyk; Wieslawa Jarmuszkiewicz; Wolfram S Kunz
Mitochondrial potassium channels.
IUBMB life 2009;61(2):134-43.
2009: Suzana Gispert; Filomena Ricciardi; Alexander Kurz; Mekhman Azizov; Hans-Hermann Hoepken; Dorothea Becker; Wolfgang Voos; Kristina Leuner; Walter E Müller; Alexei P Kudin; Wolfram S Kunz; Annabelle Zimmermann; Jochen Roeper; Dirk Wenzel; Marina Jendrach; Moisés García-Arencíbia; Javier Fernández-Ruiz; Leslie Huber; Hermann Rohrer; Miguel Barrera; Andreas S Reichert; Udo Rüb; Amy Chen; Robert L Nussbaum; Georg Auburger
Parkinson phenotype in aged PINK1-deficient mice is accompanied by progressive mitochondrial dysfunction in absence of neurodegeneration.
PloS one 2009;4(6):e5777.
2009: Dominika Malinska; Alexei P Kudin; Grazyna Debska-Vielhaber; Stefan Vielhaber; Wolfram S Kunz
Chapter 23 Quantification of superoxide production by mouse brain and skeletal muscle mitochondria.
Methods in enzymology 2009;456():419-37.
2008: Gabor Zsurka; Miriam Baron; Joanna D Stewart; Cornelia Kornblum; Monika Bös; Robert Sassen; Robert W Taylor; Christian E Elger; Patrick F Chinnery; Wolfram S Kunz
Clonally expanded mitochondrial DNA mutations in epileptic individuals with mutated DNA polymerase gamma.
Journal of neuropathology and experimental neurology 2008;67(9):857-66.
2008: Bogusz Kulawiak; Alexei P Kudin; Adam Szewczyk; Wolfram S Kunz
BK channel openers inhibit ROS production of isolated rat brain mitochondria.
Experimental neurology 2008;212(2):543-7.
2008: Sebastian Paus; Gabor Zsurka; Miriam Baron; Marcus Deschauer; Christian Bamberg; Thomas Klockgether; Wolfram S Kunz; Cornelia Kornblum
Apraxia of lid opening mimicking ptosis in compound heterozygosity for A467T and W748S POLG1 mutations.
Movement disorders : official journal of the Movement Disorder Society 2008;23(9):1286-8.
2008: Jolanta Skalska; Marta Piwońska; Elzbieta Wyroba; Liliana Surmacz; Rafal Wieczorek; Izabela Koszela-Piotrowska; Joanna Zielińska; Piotr Bednarczyk; Krzysztof Dołowy; Grzegorz M Wilczynski; Adam Szewczyk; Wolfram S Kunz
A novel potassium channel in skeletal muscle mitochondria.
Biochimica et biophysica acta 2008;1777(7-8):651-9.
2008: Alexei P Kudin; Dominika Malinska; Wolfram S Kunz
Sites of generation of reactive oxygen species in homogenates of brain tissue determined with the use of respiratory substrates and inhibitors.
Biochimica et biophysica acta 2008;1777(7-8):689-95.
2008: Cornelius F H Mueller; Kerstin Wassmann; Julian Widder; Sven Wassmann; Chia Hui Chen; Barbara Keuler; Alexey Kudin; Wolfram S Kunz; Georg Nickenig
Multidrug resistance protein-1 affects oxidative stress, endothelial dysfunction, and atherogenesis via leukotriene C4 export.
Circulation 2008;117(22):2912-8.
2008: Patryk Konieczny; Peter Fuchs; Siegfried Reipert; Wolfram S Kunz; Anikó Zeöld; Irmgard Fischer; D Paulin; Rolf Schröder; Gerhard Wiche
Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms.
The Journal of cell biology 2008;181(4):667-81.
2008: Cornelia Kornblum; Gabor Zsurka; Rudolf J Wiesner; Rolf Schröder; Wolfram S Kunz
Concerted action of two novel tRNA mtDNA point mutations in chronic progressive external ophthalmoplegia.
Bioscience reports 2008;28(2):89-96.
2008: Andrey V Kuznetsov; Vladimir Veksler; Frank N Gellerich; Valdur Saks; Raimund Margreiter; Wolfram S Kunz
Analysis of mitochondrial function in situ in permeabilized muscle fibers, tissues and cells.
Nature protocols 2008;3(6):965-76.
2008: Stefan Vielhaber; Heiko G Niessen; Grazyna Debska-Vielhaber; Alexei P Kudin; Jörg Wellmer; Jörn Kaufmann; Mircea Ariel Schönfeld; Robert Fendrich; Wieland Willker; Dieter Leibfritz; Johannes Schramm; Christian E Elger; Hans-Jochen Heinze; Wolfram S Kunz
Subfield-specific loss of hippocampal N-acetyl aspartate in temporal lobe epilepsy.
Epilepsia 2008;49(1):40-50.
2007: M Baron; Alexei P Kudin; Wolfram S Kunz
Mitochondrial dysfunction in neurodegenerative disorders.
Biochemical Society transactions 2007;35(Pt 5):1228-31.
2007: Hue-Tran Hornig-Do; Jürgen-Christoph von Kleist-Retzow; Katrin Lanz; Claudia Wickenhauser; Alexei P Kudin; Wolfram S Kunz; Rudolf J Wiesner; Matthias Schauen
Human epidermal keratinocytes accumulate superoxide due to low activity of Mn-SOD, leading to mitochondrial functional impairment.
The Journal of investigative dermatology 2007;127(5):1084-93.
2007: Heiko G Niessen; Grazyna Debska-Vielhaber; Kerstin Sander; Frank Angenstein; Albert C Ludolph; Liane Hilfert; Wieland Willker; Dieter Leibfritz; Hans-Jochen Heinze; Wolfram S Kunz; Stefan Vielhaber
Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotrophic lateral sclerosis.
The European journal of neuroscience 2007;25(6):1669-77.
2007: Gabor Zsurka; Kevin G Hampel; Tatiana A Kudina; Cornelia Kornblum; Yevgenya Kraytsberg; Christian E Elger; Konstantin Khrapko; Wolfram S Kunz
Inheritance of mitochondrial DNA recombinants in double-heteroplasmic families: potential implications for phylogenetic analysis.
American journal of human genetics 2007;80(2):298-305.
2007: Hans-Hermann Hoepken; Suzana Gispert; Blas Morales; Oliver Wingerter; Domenico Del Turco; Alexander Mülsch; Robert L Nussbaum; Klaus Müller; Stefan Dröse; Ulrich Brandt; Thomas Deller; Brunhilde Wirth; Alexei P Kudin; Wolfram S Kunz; Georg Auburger
Mitochondrial dysfunction, peroxidation damage and changes in glutathione metabolism in PARK6.
Neurobiology of disease 2007;25(2):401-11.
2007: Christian Frisch; Alexei P Kudin; Christian E Elger; Wolfram S Kunz; Christoph Helmstaedter
Amelioration of water maze performance deficits by topiramate applied during pilocarpine-induced status epilepticus is negatively dose-dependent.
Epilepsy research 2007;73(2):173-80.
2007: Izabela Koszela-Piotrowska; Katarzyna Choma; Piotr Bednarczyk; Krzysztof Dołowy; Adam Szewczyk; Wolfram S Kunz; Lubica Malekova; Viera Kominkova; Karol Ondrias
Stilbene derivatives inhibit the activity of the inner mitochondrial membrane chloride channels.
Cellular & molecular biology letters 2007;12(4):493-508.
2006: Rudolf J Wiesner; Gabor Zsurka; Wolfram S Kunz
Mitochondrial DNA damage and the aging process: facts and imaginations.
Free radical research 2006;40(12):1284-94.
2006: Heiko G Niessen; Frank Angenstein; Kerstin Sander; Wolfram S Kunz; Marko Teuchert; Albert C Ludolph; Hans-Jochen Heinze; Henning Scheich; Stefan Vielhaber
In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T2 relaxation time and apparent diffusion coefficient.
Experimental neurology 2006;201(2):293-300.
2006: Michael Linnebank; Holger Lutz; Eva Jarre; Stefan Vielhaber; Carmen Noelker; Eduard Struys; Cornelis Jakobs; Thomas Klockgether; Bernd Evert; Wolfram S Kunz; Ullrich Wüllner
Binding of copper is a mechanism of homocysteine toxicity leading to COX deficiency and apoptosis in primary neurons, PC12 and SHSY-5Y cells.
Neurobiology of disease 2006;23(3):725-30.
2006: Adam Szewczyk; Jolanta Skalska; Marta Głab; Bogusz Kulawiak; Dominika Malińska; Izabela Koszela-Piotrowska; Wolfram S Kunz
Mitochondrial potassium channels: from pharmacology to function.
Biochimica et biophysica acta 2006;1757(5-6):715-20.
2005: Stefan Vielhaber; Cornelia Kornblum; Hans-Jochen Heinze; Christian E Elger; Wolfram S Kunz
Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies--a comment.
Brain : a journal of neurology 2005;128(Pt 12):E38.
2005: Gabor Zsurka; Yevgenya Kraytsberg; Tatiana A Kudina; Cornelia Kornblum; Christian E Elger; Konstantin Khrapko; Wolfram S Kunz
Recombination of mitochondrial DNA in skeletal muscle of individuals with multiple mitochondrial DNA heteroplasmy.
Nature genetics 2005;37(8):873-7.
2005: Heiko G Niessen; Frank Angenstein; Stefan Vielhaber; Christian Frisch; Alexei P Kudin; Christian E Elger; Hans-Jochen Heinze; Henning Scheich; Wolfram S Kunz
Volumetric magnetic resonance imaging of functionally relevant structural alterations in chronic epilepsy after pilocarpine-induced status epilepticus in rats.
Epilepsia 2005;46(7):1021-6.
2005: Jolanta Skalska; Grazyna Debska; Wolfram S Kunz; Adam Szewczyk
Antidiabetic sulphonylureas activate mitochondrial permeability transition in rat skeletal muscle.
British journal of pharmacology 2005;145(6):785-91.
2005: Alexei P Kudin; Grazyna Debska-Vielhaber; Wolfram S Kunz
Characterization of superoxide production sites in isolated rat brain and skeletal muscle mitochondria.
Biomedicine & pharmacotherapy = Biomédecine & pharmacothérapie 2005;59(4):163-8.
2005: Thorsten Okulla; Wolfram S Kunz; Thomas Klockgether; Rolf Schröder; Cornelia Kornblum
Diagnostic value of mitochondrial DNA mutation analysis in juvenile unilateral ptosis.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie 2005;243(4):380-2.
2005: Kirstin Winkler-Stuck; Elmar Kirches; Christian Mawrin; Knut Dietzmann; Hartmut Lins; Claus-Werner Wallesch; Wolfram S Kunz; Falk R Wiedemann
Re-evaluation of the dysfunction of mitochondrial respiratory chain in skeletal muscle of patients with Parkinson's disease.
Journal of neural transmission (Vienna, Austria : 1996) 2005;112(4):499-518.
2005: Martina Minnerop; Cornelia Kornblum; Alexius Y Joe; K Tatsch; Wolfram S Kunz; Thomas Klockgether; Ullrich Wüllner; Michael Reinhardt
Dopamine transporter SPECT in patients with mitochondrial disorders.
Journal of neurology, neurosurgery, and psychiatry 2005;76(1):118-20.
2004: Cornelia Kornblum; Wolfram S Kunz; Thomas Klockgether; Peter Roggenkämper; Rolf Schröder
[Diagnostic value of mitochondrial DNA analysis in chronic progressive external ophthalmoplegia (CPEO)].
Klinische Monatsblätter für Augenheilkunde 2004;221(12):1057-61.
2004: Gabor Zsurka; Rolf Schröder; Cornelia Kornblum; J Rudolph; Rudolf J Wiesner; Christian E Elger; Wolfram S Kunz
Tissue dependent co-segregation of the novel pathogenic G12276A mitochondrial tRNALeu(CUN) mutation with the A185G D-loop polymorphism.
Journal of medical genetics 2004;41(12):e124.
2004: Alexei P Kudin; Grazyna Debska-Vielhaber; Stefan Vielhaber; Christian E Elger; Wolfram S Kunz
The mechanism of neuroprotection by topiramate in an animal model of epilepsy.
Epilepsia 2004;45(12):1478-87.
2004: Stefan Vielhaber; Helmut Feistner; J Weis; J Kreuder; Michael Sailer; J M Schröder; Wolfram S Kunz
Primary carnitine deficiency: adult onset lipid storage myopathy with a mild clinical course.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2004;11(8):919-24.
2004: Kirstin Winkler-Stuck; Falk R Wiedemann; Claus-Werner Wallesch; Wolfram S Kunz
Effect of coenzyme Q10 on the mitochondrial function of skin fibroblasts from Parkinson patients.
Journal of the neurological sciences 2004;220(1-2):41-8.
2004: Yevgenya Kraytsberg; Marianne Schwartz; Timothy Brown; Konstantin Ebralidse; Wolfram S Kunz; David A Clayton; John Vissing; Konstantin Khrapko
Recombination of human mitochondrial DNA.
Science (New York, N.Y.) 2004;304(5673):981.
2004: Alexei P Kudin; Nana Yaw-B Bimpong-Buta; Stefan Vielhaber; Christian E Elger; Wolfram S Kunz
Characterization of superoxide-producing sites in isolated brain mitochondria.
The Journal of biological chemistry 2004;279(6):4127-35.
2004: Wolfram S Kunz; Nana Yaw-B Bimpong-Buta; Alexei P Kudin; Christian E Elger
The role of mitochondria in epilepsy: implications for neurodegenerative diseases.
Toxicology mechanisms and methods 2004;14(1-2):19-23.
2003: Stefan Vielhaber; Alexei P Kudin; Tatiana A Kudina; Detlef Stiller; Henning Scheich; Ariel Schoenfeld; Helmut Feistner; Hans-Jochen Heinze; Christian E Elger; Wolfram S Kunz
Hippocampal N-acetyl aspartate levels do not mirror neuronal cell densities in creatine-supplemented epileptic rats.
The European journal of neuroscience 2003;18(8):2292-300.
2003: Andrey V Kuznetsov; Wolfram S Kunz; Valdur Saks; Yves Usson; Jean-Pierre Mazat; Thierry Letellier; Frank N Gellerich; Raimund Margreiter
Cryopreservation of mitochondria and mitochondrial function in cardiac and skeletal muscle fibers.
Analytical biochemistry 2003;319(2):296-303.
2003: Stefan Vielhaber; Alexei P Kudin; Kirstin Winkler; Falk R Wiedemann; Rolf Schröder; Helmut Feistner; Hans-Jochen Heinze; Christian E Elger; Wolfram S Kunz
Is there mitochondrial dysfunction in amyotrophic lateral sclerosis skeletal muscle?
Annals of neurology 2003;53(5):686-7; author reply 687-8.
2003: Rolf Schröder; Bertrand Goudeau; Monique Casteras Simon; Dirk Fischer; Thomas Eggermann; Christoph S Clemen; Zhenlin Li; Jens Reimann; Zhigang Xue; Sabine Rudnik-Schöneborn; Klaus Zerres; Peter F.M. van der Ven; Dieter O Fürst; Wolfram S Kunz; Patrick Vicart
On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria.
Human molecular genetics 2003;12(6):657-69.
2003: Jens Reimann; Wolfram S Kunz; Stefan Vielhaber; Karin Kappes-Horn; Rolf Schröder
Mitochondrial dysfunction in myofibrillar myopathy.
Neuropathology and applied neurobiology 2003;29(1):45-51.
2003: Stefan Vielhaber; Joachim von Oertzen; Alexei F Kudin; Ariel Schoenfeld; Christian Menzel; Hans-Jürgen Biersack; Thomas Kral; Christian E Elger; Wolfram S Kunz
Correlation of hippocampal glucose oxidation capacity and interictal FDG-PET in temporal lobe epilepsy.
Epilepsia 2003;44(2):193-9.
2003: Jürgen-Christoph von Kleist-Retzow; Ulrike Schauseil-Zipf; Dietrich V Michalk; Wolfram S Kunz
Mitochondrial diseases--an expanding spectrum of disorders and affected genes.
Experimental physiology 2003;88(1):155-66.
2003: Wolfram S Kunz
Different metabolic properties of mitochondrial oxidative phosphorylation in different cell types--important implications for mitochondrial cytopathies.
Experimental physiology 2003;88(1):149-54.
2002: Grazyna Debska; Anna Kicinska; Jolanta Skalska; Adam Szewczyk; Rebecca May; Christian E Elger; Wolfram S Kunz
Opening of potassium channels modulates mitochondrial function in rat skeletal muscle.
Biochimica et biophysica acta 2002;1556(2-3):97-105.
2002: Stefan Vielhaber; Dmitry A Varlamov; Tatiana A Kudina; Rolf Schröder; Karin Kappes-Horn; Christian E Elger; Martina Seibel; Peter Seibel; Wolfram S Kunz
Expression pattern of mitochondrial respiratory chain enzymes in skeletal muscle of patients harboring the A3243G point mutation or large-scale deletions of mitochondrial DNA.
Journal of neuropathology and experimental neurology 2002;61(10):885-95.
2002: Dmitry A Varlamov; Alexei P Kudin; Stefan Vielhaber; Rolf Schröder; Robert Sassen; Albert Becker; Dagmar Kunz; Karsten Haug; Johannes Rebstock; Armin Heils; Christian E Elger; Wolfram S Kunz
Metabolic consequences of a novel missense mutation of the mtDNA CO I gene.
Human molecular genetics 2002;11(16):1797-805.
2002: Rolf Schröder; Wolfram S Kunz; Fatima Rouan; Ellen Pfendner; Karen Tolksdorf; Karin Kappes-Horn; Manuela Altenschmidt-Mehring; Rupert Knoblich; Peter F.M. van der Ven; Jens Reimann; Dieter O Fürst; Ingmar Blümcke; Stefan Vielhaber; Detlef Zillikens; Sabine A Eming; Thomas Klockgether; Jouni Uitto; Gerhard Wiche; Arndt Rolfs
Disorganization of the desmin cytoskeleton and mitochondrial dysfunction in plectin-related epidermolysis bullosa simplex with muscular dystrophy.
Journal of neuropathology and experimental neurology 2002;61(6):520-30.
2002: Alexei P Kudin; Tatiana A Kudina; Jan Seyfried; Stefan Vielhaber; Heinz Beck; Christian E Elger; Wolfram S Kunz
Seizure-dependent modulation of mitochondrial oxidative phosphorylation in rat hippocampus.
The European journal of neuroscience 2002;15(7):1105-14.
2002: Wolfram S Kunz
The role of mitochondria in epileptogenesis.
Current opinion in neurology 2002;15(2):179-84.
2002: Alexei P Kudin; Stefan Vielhaber; Christian E Elger; Wolfram S Kunz
Differences in flux control and reserve capacity of cytochrome c oxidase (COX) in human skeletal muscle and brain suggest different metabolic effects of mild COX deficiencies.
Molecular biology reports 2002;29(1-2):89-92.
2002: Dagmar Kunz; Kirstin Winkler; Christian E Elger; Wolfram S Kunz
Functional imaging of mitochondrial redox state.
Methods in enzymology 2002;352():135-50.
2001: Stefan Vielhaber; Jörn Kaufmann; Martin Kanowski; Michael Sailer; Helmut Feistner; Claus Tempelmann; Christian E Elger; Hans-Jochen Heinze; Wolfram S Kunz
Effect of creatine supplementation on metabolite levels in ALS motor cortices.
Experimental neurology 2001;172(2):377-82.
2001: Stefan Vielhaber; Rolf Schröder; Kirstin Winkler; S Weis; Michael Sailer; Helmut Feistner; H J Heinze; J M Schröder; Wolfram S Kunz
Defective mitochondrial oxidative phosphorylation in myopathies with tubular aggregates originating from sarcoplasmic reticulum.
Journal of neuropathology and experimental neurology 2001;60(11):1032-40.
2001: Wolfram S Kunz
Control of oxidative phosphorylation in skeletal muscle.
Biochimica et biophysica acta 2001;1504(1):12-9.
2001: Grazyna Debska; R May; A Kicińska; Adam Szewczyk; Christian E Elger; Wolfram S Kunz
Potassium channel openers depolarize hippocampal mitochondria.
Brain research 2001;892(1):42-50.
2001: Cordelia S Schwarz; Bernd Evert; Jan Seyfried; M Schaupp; Wolfram S Kunz; Stefan Vielhaber; Thomas Klockgether; Ullrich Wüllner
Overexpression of bcl-2 results in reduction of cytochrome c content and inhibition of complex I activity.
Biochemical and biophysical research communications 2001;280(4):1021-7.
2000: Armin Heils; K Haug; Wolfram S Kunz; G Fernandez; Steve Horvath; Johannes Rebstock; Peter Propping; Christian E Elger
Interleukin-1beta gene polymorphism and susceptibility to temporal lobe epilepsy with hippocampal sclerosis.
Annals of neurology 2000;48(6):948-50.
2000: Wolfram S Kunz; Alexei P Kudin; Stefan Vielhaber; Ingmar Blümcke; W Zuschratter; Johannes Schramm; Heinz Beck; Christian E Elger
Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy.
Annals of neurology 2000;48(5):766-73.
2000: Wolfram S Kunz; Alexei P Kudin; Stefan Vielhaber; Christian E Elger; Giuseppe Attardi; Gaetano Villani
Flux control of cytochrome c oxidase in human skeletal muscle.
The Journal of biological chemistry 2000;275(36):27741-5.
2000: Stefan Vielhaber; D Kunz; Kirstin Winkler; Falk R Wiedemann; Elmar Kirches; Helmut Feistner; Hans-Jochen Heinze; Christian E Elger; W Schubert; Wolfram S Kunz
Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis.
Brain : a journal of neurology 2000;123 ( Pt 7)():1339-48.
2000: Rolf Schröder; Stefan Vielhaber; Falk R Wiedemann; Cornelia Kornblum; Andreas Papassotiropoulos; P Broich; S Zierz; Christian E Elger; Heinz Reichmann; Peter Seibel; Thomas Klockgether; Wolfram S Kunz
New insights into the metabolic consequences of large-scale mtDNA deletions: a quantitative analysis of biochemical, morphological, and genetic findings in human skeletal muscle.
Journal of neuropathology and experimental neurology 2000;59(5):353-60.
2000: Jan Seyfried; F Soldner; Wolfram S Kunz; Jörg B Schulz; Thomas Klockgether; Karl-Artur Kovar; Ullrich Wüllner
Effect of 1-methyl-4-phenylpyridinium on glutathione in rat pheochromocytoma PC 12 cells.
Neurochemistry international 2000;36(6):489-97.
2000: Falk R Wiedemann; Stefan Vielhaber; Rolf Schröder; Christian E Elger; Wolfram S Kunz
Evaluation of methods for the determination of mitochondrial respiratory chain enzyme activities in human skeletal muscle samples.
Analytical biochemistry 2000;279(1):55-60.
2000: Stefan Vielhaber; Alexei P Kudin; Rolf Schröder; Christian E Elger; Wolfram S Kunz
Muscle fibres: applications for the study of the metabolic consequences of enzyme deficiencies in skeletal muscle.
Biochemical Society transactions 2000;28(2):159-64.
2000: Stefan Vielhaber; Helmut Feistner; W Schneider; J Weis; Wolfram S Kunz
Mitochondrial complex I deficiency in a female with multiplex arthrogryposis congenita.
Pediatric neurology 2000;22(1):53-6.
1999: Alexei P Kudin; Stefan Vielhaber; Heinz Beck; Christian E Elger; Wolfram S Kunz
Quantitative investigation of mitochondrial function in single rat hippocampal slices: a novel application of high-resolution respirometry and laser-excited fluorescence spectroscopy.
Brain research. Brain research protocols 1999;4(3):329-34.
1999: Stefan Vielhaber; K Winkler; Elmar Kirches; D Kunz; Maren Büchner; Helmut Feistner; Christian E Elger; Albert C Ludolph; M W Riepe; Wolfram S Kunz
Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis.
Journal of the neurological sciences 1999;169(1-2):133-9.
1999: Elmar Kirches; Kirstin Winkler; Stefan Vielhaber; Matthias Michael; M Warich-Kirches; P Von Bossanyi; Ines Plate; Wolfram S Kunz; Reinhard Szibor; Helmut Feistner; Knut Dietzmann
Mitochondrial tRNA(Cys) mutation A5823G in a patient with motor neuron disease and temporal lobe epilepsy.
Pathobiology : journal of immunopathology, molecular and cellular biology 1999;67(4):214-8.
1999: Wolfram S Kunz; IV Goussakov; Heinz Beck; Christian E Elger
Altered mitochondrial oxidative phosphorylation in hippocampal slices of kainate-treated rats.
Brain research 1999;826(2):236-42.
1999: Wolfram S Kunz; A V Kuznetsov; Joseph F Clark; I Tracey; Christian E Elger
Metabolic consequences of the cytochrome c oxidase deficiency in brain of copper-deficient Mo(vbr) mice.
Journal of neurochemistry 1999;72(4):1580-5.
1999: Falk R Wiedemann; Kirstin Winkler; Hartmut Lins; Claus-Werner Wallesch; Wolfram S Kunz
Detection of respiratory chain defects in cultivated skin fibroblasts and skeletal muscle of patients with Parkinson's disease.
Annals of the New York Academy of Sciences 1999;893():426-9.
1998: Valdur Saks; V I Veksler; Andrey V Kuznetsov; Laurence Kay; Peeter Sikk; Toomas Tiivel; Leone Tranqui; Jose Olivares; Kirstin Winkler; Falk R Wiedemann; Wolfram S Kunz
Permeabilized cell and skinned fiber techniques in studies of mitochondrial function in vivo.
Molecular and cellular biochemistry 1998;184(1-2):81-100.
1998: Elmar Kirches; Kirstin Winkler; M Warich-Kirches; Reinhard Szibor; F Wien; Wolfram S Kunz; P Von Bossanyi; P K Bajaj; Knut Dietzmann
mtDNA depletion and impairment of mitochondrial function in a case of a multisystem disorder including severe myopathy.
Journal of inherited metabolic disease 1998;21(4):400-8.
1998: AV Kuznetsov; Kirstin Winkler; Falk R Wiedemann; P Von Bossanyi; Knut Dietzmann; Wolfram S Kunz
Impaired mitochondrial oxidative phosphorylation in skeletal muscle of the dystrophin-deficient mdx mouse.
Molecular and cellular biochemistry 1998;183(1-2):87-96.
1998: AV Kuznetsov; Oleg Mayboroda; D Kunz; Kirstin Winkler; W Schubert; Wolfram S Kunz
Functional imaging of mitochondria in saponin-permeabilized mice muscle fibers.
The Journal of cell biology 1998;140(5):1091-9.
1998: Falk R Wiedemann; Wolfram S Kunz
Oxygen dependence of flux control of cytochrome c oxidase -- implications for mitochondrial diseases.
FEBS letters 1998;422(1):33-5.
1998: AV Kuznetsov; Falk R Wiedemann; Kirstin Winkler; Wolfram S Kunz
Use of saponin-permeabilized muscle fibers for the diagnosis of mitochondrial diseases.
BioFactors (Oxford, England) 1998;7(3):221-3.
1998: Falk R Wiedemann; Kirstin Winkler; AV Kuznetsov; C Bartels; Stefan Vielhaber; Helmut Feistner; Wolfram S Kunz
Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis.
Journal of the neurological sciences 1998;156(1):65-72.
1997: Wolfram S Kunz; Kirstin Winkler; AV Kuznetsov; Hartmut Lins; Elmar Kirches; Claus-Werner Wallesch
Detection of mitochondrial defects by laser fluorimetry.
Molecular and cellular biochemistry 1997;174(1-2):97-100.
1997: AV Kuznetsov; Kirstin Winkler; Elmar Kirches; Hartmut Lins; Helmut Feistner; Wolfram S Kunz
Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases.
Biochimica et biophysica acta 1997;1360(2):142-50.
1997: D Kunz; C Luley; Kirstin Winkler; Hartmut Lins; Wolfram S Kunz
Flow cytometric detection of mitochondrial dysfunction in subpopulations of human mononuclear cells.
Analytical biochemistry 1997;246(2):218-24.
1996: AV Kuznetsov; Joseph F Clark; Kirstin Winkler; Wolfram S Kunz
Increase of flux control of cytochrome c oxidase in copper-deficient mottled brindled mice.
The Journal of biological chemistry 1996;271(1):283-8.
1995: Kirstin Winkler; AV Kuznetsov; Hartmut Lins; Elmar Kirches; P Von Bossanyi; Knut Dietzmann; B Frank; Helmut Feistner; Wolfram S Kunz
Laser-excited fluorescence studies of mitochondrial function in saponin-skinned skeletal muscle fibers of patients with chronic progressive external ophthalmoplegia.
Biochimica et biophysica acta 1995;1272(3):181-4.
1995: E Wisniewski; Frank N Gellerich; Wolfram S Kunz
Distribution of flux control among the enzymes of mitochondrial oxidative phosphorylation in calcium-activated saponin-skinned rat musculus soleus fibers.
European journal of biochemistry / FEBS 1995;230(2):549-54.
1995: D Kunz; Claus Luley; S Fritz; Ralf Bohnensack; Kirstin Winkler; Wolfram S Kunz; Claus-Werner Wallesch
Oxygraphic evaluation of mitochondrial function in digitonin-permeabilized mononuclear cells and cultured skin fibroblasts of patients with chronic progressive external ophthalmoplegia.
Biochemical and molecular medicine 1995;54(2):105-11.
1994: Z Khuchua; Y Belikova; AV Kuznetsov; Frank N Gellerich; Lorenz Schild; Hans-Wolfram Neumann; Wolfram S Kunz
Caffeine and Ca2+ stimulate mitochondrial oxidative phosphorylation in saponin-skinned human skeletal muscle fibers due to activation of actomyosin ATPase.
Biochimica et biophysica acta 1994;1188(3):373-9.
1994: Wolfram S Kunz; AV Kuznetsov; K Winkler; Frank N Gellerich; S Neuhof; Hans-Wolfram Neumann
Measurement of fluorescence changes of NAD(P)H and of fluorescent flavoproteins in saponin-skinned human skeletal muscle fibers.
Analytical biochemistry 1994;216(2):322-7.
1993: Wolfram S Kunz; AV Kuznetsov; W Schulze; K Eichhorn; Lorenz Schild; F Striggow; Ralf Bohnensack; S Neuhof; Henning Grasshoff; H W Neumann; F N Gellerich
Functional characterization of mitochondrial oxidative phosphorylation in saponin-skinned human muscle fibers.
Biochimica et biophysica acta 1993;1144(1):46-53.
1993: Wolfram S Kunz; Frank N Gellerich
Quantification of the content of fluorescent flavoproteins in mitochondria from liver, kidney cortex, skeletal muscle, and brain.
Biochemical medicine and metabolic biology 1993;50(1):103-10.
1993: Wolfram S Kunz; AV Kuznetsov; Frank N Gellerich
Mitochondrial oxidative phosphorylation in saponin-skinned human muscle fibers is stimulated by caffeine.
FEBS letters 1993;323(1-2):188-90.
1993: E Wisniewski; Wolfram S Kunz; Frank N Gellerich
Phosphate affects the distribution of flux control among the enzymes of oxidative phosphorylation in rat skeletal muscle mitochondria.
The Journal of biological chemistry 1993;268(13):9343-6.
1992: Wolfram S Kunz; L Krinelke; Frank N Gellerich
Glutamine affects glutamate metabolism in isolated rat kidney cortex mitochondria.
Biochimica et biophysica acta 1992;1100(3):329-31.
1992: Heiko Noack; Wolfram S Kunz; Wolfgang Augustin
Evaluation of a procedure for the simultaneous determination of oxidized and reduced pyridine nucleotides and adenylates in organic phenol extracts from mitochondria.
Analytical biochemistry 1992;202(1):162-5.
1991: Walter Halangk; Wolfram S Kunz
Use of NAD(P)H and flavoprotein fluorescence signals to characterize the redox state of pyridine nucleotides in epididymal bull spermatozoa.
Biochimica et biophysica acta 1991;1056(3):273-8.
1991: Wolfram S Kunz
Application of the theory of steady-state flux control to mitochondrial beta-oxidation.
Biomedica biochimica acta 1991;50(12):1143-57.
1990: Frank N Gellerich; Wolfram S Kunz; Ralf Bohnensack
Estimation of flux control coefficients from inhibitor titrations by non-linear regression.
FEBS letters 1990;274(1-2):167-70.
1990: L Krinelke; Wolfram S Kunz
[Characterization of surface fluorescence signals of isolated perfused rat kidney].
Biomedica biochimica acta 1990;49(11):1119-30.
1988: Wolfram S Kunz
Evaluation of electron-transfer flavoprotein and alpha-lipoamide dehydrogenase redox states by two-channel fluorimetry and its application to the investigation of beta-oxidation.
Biochimica et biophysica acta 1988;932(1):8-16.