Zobrazeno 1 - 8
of 8
pro vyhledávání: '"Laura P Pietikäinen"'
Publikováno v:
PLoS ONE, Vol 8, Iss 1, p e53688 (2013)
Molecular basis of D-bifunctional protein (D-BP) deficiency was studied with wild type and five disease-causing variants of 3R-hydroxyacyl-CoA dehydrogenase fragment of the human MFE-2 (multifunctional enzyme type 2) protein. Complementation analysis
Externí odkaz:
https://doaj.org/article/a4b1ec5485804990a6b27f3921a68a2f
Autor:
Laura P. Pietikäinen, M. Tanvir Rahman, J. Kalervo Hiltunen, Carol L. Dieckmann, Alexander J. Kastaniotis
Publikováno v:
BMC Biology, Vol 19, Iss 1, Pp 1-15 (2021)
Abstract Background Lipoylation of 2-ketoacid dehydrogenases is essential for mitochondrial function in eukaryotes. While the basic principles of the lipoylation processes have been worked out, we still lack a thorough understanding of the details of
Externí odkaz:
https://doaj.org/article/f012e0c5783342f1922b0a5e5a5651a5
Autor:
J.K. Hiltunen, Carol L. Dieckmann, M. T. Rahman, Alexander J. Kastaniotis, Laura P. Pietikäinen
Publikováno v:
BMC Biology
BMC Biology, Vol 19, Iss 1, Pp 1-15 (2021)
BMC Biology, Vol 19, Iss 1, Pp 1-15 (2021)
Background Lipoylation of 2-ketoacid dehydrogenases is essential for mitochondrial function in eukaryotes. While the basic principles of the lipoylation processes have been worked out, we still lack a thorough understanding of the details of this imp
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c26e0d89e9f3efbc931afbfe306ca602
https://doi.org/10.1186/s12915-021-00951-3
https://doi.org/10.1186/s12915-021-00951-3
Autor:
Geoffray Monteuuis, Anne M. Mäkelä, J. Kalervo Hiltunen, Remya R. Nair, Kaija J. Autio, Antonina Shvetsova, Laura P. Pietikäinen, Alexander J. Kastaniotis, Zhijun Chen, Juha M. Kerätär
Publikováno v:
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids. 1862:39-48
Mitochondria and fatty acids are tightly connected to a multiplicity of cellular processes that go far beyond mitochondrial fatty acid metabolism. In line with this view, there is hardly any common metabolic disorder that is not associated with distu
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cce035a768188694052192a6fc5a0cff
https://doi.org/10.1016/j.bbalip.2016.08.011
https://doi.org/10.1016/j.bbalip.2016.08.011
Autor:
Gali Heimer, Juha M. Kerätär, Lisa G. Riley, Shanti Balasubramaniam, Eran Eyal, Laura P. Pietikäinen, J. Kalervo Hiltunen, Dina Marek-Yagel, Jeffrey Hamada, Allison Gregory, Caleb Rogers, Penelope Hogarth, Martha A. Nance, Nechama Shalva, Alvit Veber, Michal Tzadok, Andreea Nissenkorn, Davide Tonduti, Florence Renaldo, Ichraf Kraoua, Celeste Panteghini, Lorella Valletta, Barbara Garavaglia, Mark J. Cowley, Velimir Gayevskiy, Tony Roscioli, Jonathon M. Silberstein, Chen Hoffmann, Annick Raas-Rothschild, Valeria Tiranti, Yair Anikster, John Christodoulou, Alexander J. Kastaniotis, Bruria Ben-Zeev, Susan J. Hayflick, Michael J. Bamshad, Suzanne M. Leal, Deborah A. Nickerson, Peter Anderson, Marcus Annable, Elizabeth Marchani Blue, Kati J. Buckingham, Jennifer Chin, Jessica X. Chong, Rodolfo Cornejo, Colleen P. Davis, Christopher Frazar, Zongxiao He, Gail P. Jarvik, Guillaume Jimenez, Eric Johanson, Tom Kolar, Stephanie A. Krauter, Daniel Luksic, Colby T. Marvin, Sean McGee, Daniel J. McGoldrick, Karynne Patterson, Marcos Perez, Sam W. Phillips, Jessica Pijoan, Peggy D. Robertson, Regie Santos-Cortez, Aditi Shankar, Krystal Slattery, Kathryn M. Shively, Deborah L. Siegel, Joshua D. Smith, Monica Tackett, Gao Wang, Marc Wegener, Jeffrey M. Weiss, Riana I. Wernick, Marsha M. Wheeler, Qian Yi
Publikováno v:
The American Journal of Human Genetics. 99:1229-1244
Mitochondrial fatty acid synthesis (mtFAS) is an evolutionarily conserved pathway essential for the function of the respiratory chain and several mitochondrial enzyme complexes. We report here a unique neurometabolic human disorder caused by defectiv
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8f5df4ba7c05bbdf056967b31831bace
https://doi.org/10.1016/j.ajhg.2016.09.021
https://doi.org/10.1016/j.ajhg.2016.09.021
Autor:
Mari J. Aaltonen, Remya R. Nair, Melissa S. Schonauer, Laura P. Pietikäinen, V. A. Samuli Kursu, J. Kalervo Hiltunen, Carol L. Dieckmann, Alexander J. Kastaniotis, Flavia Fontanesi, Antoni Barrientos, Fumi Suomi
Publikováno v:
Molecular Microbiology. 90:824-840
Summary Mitochondrial fatty acid synthesis (mtFAS) shares acetyl-CoA with the Krebs cycle as a common substrate and is required for the production of octanoic acid (C8) precursors of lipoic acid (LA) in mitochondria. MtFAS is a conserved pathway esse
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::28141cda7321d30da2cd4cfd4f0fa66e
https://doi.org/10.1111/mmi.12402
https://doi.org/10.1111/mmi.12402
Publikováno v:
PLoS ONE, Vol 8, Iss 1, p e53688 (2013)
PLoS ONE
PLoS ONE, 2013, 8, pp.e53688. ⟨10.1371/journal.pone.0053688⟩
PLoS ONE, Public Library of Science, 2013, 8, pp.e53688. ⟨10.1371/journal.pone.0053688⟩
PLoS ONE
PLoS ONE, 2013, 8, pp.e53688. ⟨10.1371/journal.pone.0053688⟩
PLoS ONE, Public Library of Science, 2013, 8, pp.e53688. ⟨10.1371/journal.pone.0053688⟩
Molecular basis of D-bifunctional protein (D-BP) deficiency was studied with wild type and five disease-causing variants of 3R-hydroxyacyl-CoA dehydrogenase fragment of the human MFE-2 (multifunctional enzyme type 2) protein. Complementation analysis
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::39b077465f3ad10356ec54a34bf54f79
http://europepmc.org/articles/PMC3538638?pdf=render
http://europepmc.org/articles/PMC3538638?pdf=render
Publikováno v:
Acta Crystallographica Section A Foundations of Crystallography. 65:s142-s142
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::9ea65643f9509636e34fc65e193ebb23
https://doi.org/10.1107/s0108767309097153
https://doi.org/10.1107/s0108767309097153