Zobrazeno 1 - 10
of 37
pro vyhledávání: '"Jürgen Christoph Von Kleist-Retzow"'
Autor:
Andras Franko, Martin Irmler, Cornelia Prehn, Silke S. Heinzmann, Philippe Schmitt-Kopplin, Jerzy Adamski, Johannes Beckers, Jürgen-Christoph von Kleist-Retzow, Rudolf Wiesner, Hans-Ulrich Häring, Martin Heni, Andreas L. Birkenfeld, Martin Hrabě de Angelis
Publikováno v:
Biomedicines, Vol 10, Iss 3, p 616 (2022)
Glucotoxic metabolites and pathways play a crucial role in diabetic complications, and new treatment options which improve glucotoxicity are highly warranted. In this study, we analyzed bezafibrate (BEZ) treated, streptozotocin (STZ) injected mice, w
Externí odkaz:
https://doaj.org/article/b41a9e1a191b4428813e24dc91366f22
Autor:
Andras Franko, Martin Irmler, Cornelia Prehn, Silke S. Heinzmann, Philippe Schmitt-Kopplin, Jerzy Adamski, Johannes Beckers, Jürgen-Christoph von Kleist-Retzow, Rudolf Wiesner, Hans-Ulrich Häring, Martin Heni, Andreas L. Birkenfeld, Martin Hrabě de Angelis
Publikováno v:
Biomedicines 10:616 (2022)
Biomedicines; Volume 10; Issue 3; Pages: 616
Biomedicines; Volume 10; Issue 3; Pages: 616
Glucotoxic metabolites and pathways play a crucial role in diabetic complications, and new treatment options which improve glucotoxicity are highly warranted. In this study, we analyzed bezafibrate (BEZ) treated, streptozotocin (STZ) injected mice, w
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4778f0e6bd866c68c810c3ce0355be69
https://mediatum.ub.tum.de/doc/1657636/document.pdf
https://mediatum.ub.tum.de/doc/1657636/document.pdf
Autor:
Andras Franko, Alexander Kunze, Marlen Böse, Jürgen-Christoph von Kleist-Retzow, Mats Paulsson, Ursula Hartmann, Rudolf J. Wiesner
Publikováno v:
International Journal of Molecular Sciences, Vol 18, Iss 6, p 1156 (2017)
Mitochondria play a pivotal role in energy metabolism, but whether insulin signaling per se could regulate mitochondrial function has not been identified yet. To investigate whether mitochondrial function is regulated by insulin signaling, we analyze
Externí odkaz:
https://doaj.org/article/0ccb52277b664d86b7a986eb19c53d22
Autor:
Joachim Kreuder, Norbert Graf, Hans Martin Bosse, Thomas Fischbach, Martin Häusler, Jürgen-Christoph von Kleist-Retzow
Publikováno v:
Monatsschrift Kinderheilkunde 169(2), 151-158 (2021). doi:10.1007/s00112-020-01076-7 special issue: "Pädiatrische Dermatologie"
Monatsschrift Kinderheilkunde 169(2), 151-158 (2021). doi:10.1007/s00112-020-01076-7 special issue: "Pädiatrische Dermatologie"
Published by Springer, Berlin
Published by Springer, Berlin
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3f428a029c128e5c50309ff941a76a5d
Autor:
Brunhilde Wirth, Mary K. Schroth, Sandra P. Reyna, Kathryn J. Swoboda, Ren Zhe Zhang, Charles B. Scott, Kristin J. Krosschell, Thomas O. Crawford, Elise L. Townsend, Priya S. Kishnani, John T. Kissel, Bernie LaSalle, Jürgen-Christoph von Kleist-Retzow, Edward C. Smith, Thomas W. Prior, Sarah D Simeone, Louise R. Simard, Barbara Hero, Guy D'Anjou, Bakri Elsheikh, Gyula Acsadi, Ai Sakonju
Publikováno v:
Muscle & Nerve. 57:193-199
Introduction:To explore safety and therapeutic potential of L-carnitine and valproic acid (VPA) in infants with spinal muscular atrophy (SMA). Methods: Open-label phase 2 multicenter trial of L-carnitine and VPA in infants with SMA type I with retros
Autor:
Kristin J, Krosschell, John T, Kissel, Elise L, Townsend, Sarah D, Simeone, Ren Zhe, Zhang, Sandra P, Reyna, Thomas O, Crawford, Mary K, Schroth, Gyula, Acsadi, Priya S, Kishnani, Jürgen-Christoph, Von Kleist-Retzow, Barbara, Hero, Guy, D'Anjou, Edward C, Smith, Bakri, Elsheikh, Louise R, Simard, Thomas W, Prior, Charles B, Scott, Bernard, Lasalle, Ai, Sakonju, Brunhilde, Wirth, Kathryn J, Swoboda
Publikováno v:
Musclenerve. 57(2)
The aim of this study was to determine the safety and therapeutic potential of L-carnitine and valproic acid (VPA) in infants with spinal muscular atrophy (SMA).Our investigation was an open-label phase 2 multicenter trial of L-carnitine and VPA in i
Autor:
Christian Mielke, Heiner Schaal, Frank Hillebrand, Rudolf J. Wiesner, Ilaria Dalla Rosa, Fritz Boege, Morten O. Christensen, Stefan Sobek, Hongliang Zhang, Faiza Kalfalah, Beatrice Bornholz, Yves Pommier, Jürgen-Christoph von Kleist-Retzow
Publikováno v:
Nucleic Acids Research
Mitochondrial topoisomerase I is a genetically distinct mitochondria-dedicated enzyme with a crucial but so far unknown role in the homeostasis of mitochondrial DNA metabolism. Here, we present data suggesting a negative regulatory function in mitoch
Autor:
Patrick F. Chinnery, Carlo Dionisi-Vici, Adrian C. Sewell, Barbara Plecko, Jose M. Trejo-Gabriel-Galán, Ute Spiekerkoetter, Jürgen Christoph von Kleist-Retzow, Eugen Mengel, Patricie Burda, Megumi Tsuji, Regina Mulder-Bleile, Maria Antonia Vilaseca, Beat Steinmann, Brian Fowler, Rossella Parini, Alexander Lossos, Gülden Gökçay, Valerie Walker, Mübeccel Demirkol, Johannes Häberle, D. Sean Froese, Dariusz Rokicki, Andrew A. M. Morris, Bruria Ben Zeev, Grazia Taddeucci, Dries Dobbelaere, Jiri Zeman, Terttu Suormala, Wolfgang Sperl, Friedrich K. Trefz, Matthias R. Baumgartner, Mareike Schimmel, K. E. Niezen-Koning, Manuel Schiff, Martina Huemer
Publikováno v:
JOURNAL OF INHERITED METABOLIC DISEASE
r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
instname
r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Fundació Sant Joan de Déu
Journal of inherited metabolic disease
Journal of Inherited Metabolic Disease, 39(1), 115-124. SPRINGER
r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
instname
r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Fundació Sant Joan de Déu
Journal of inherited metabolic disease
Journal of Inherited Metabolic Disease, 39(1), 115-124. SPRINGER
BACKGROUND: Severe methylenetetrahydrofolate reductase (MTHFR) deficiency is a rare (1.5% control) in vitro residual enzyme activity had mainly psychiatric symptoms, mental retardation, myelopathy, ataxia and spasticity. Treatment with various combin
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dde7d103e5d38e4744894aa67edb4b54
http://fundanet.fsjd.org/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=13399
http://fundanet.fsjd.org/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=13399
Autor:
Georgia Vlahou, Jürgen-Christoph von Kleist-Retzow, Rudolf J. Wiesner, Marek Eliáš, Francisco Rivero
Publikováno v:
European Journal of Cell Biology. 90:342-355
Ras-related GTPases of the Miro family have been implicated in mitochondrial homeostasis and microtubule-dependent transport. They consist of two GTP-binding domains separated by calcium-binding motifs and of a C-terminal transmembrane domain that ta
Autor:
Martin Lammens, Merei Huigsloot, Richard J. Rodenburg, Radek Szklarczyk, Ute Spiekerkoetter, Lambert P. van den Heuvel, Martijn A. Huynen, Werner J.H. Koopman, Jürgen-Christoph von Kleist-Retzow, Marleen Forkink, Jack A.M. Fransen, Jitske Jansen, An I. Jonckheere, Jan A.M. Smeitink
Publikováno v:
Mitochondrion, 11, 6, pp. 954-63
Mitochondrion, 11, 954-63
ResearcherID
Mitochondrion, 11, 954-63
ResearcherID
Contains fulltext : 97316.pdf (Publisher’s version ) (Closed access) We report a fragmented mitochondrial network and swollen and irregularly shaped mitochondria with partial to complete loss of the cristae in fibroblasts of a patient with a novel