Increased antioxidant response in medium-chain acyl-CoA dehydrogenase deficiency : does lipoic acid have a protective role? / Nochi, Zahra; Birkler, Rune Isak Dupont; Fernandez-Guerra, Paula; Hansen, Jakob; Wibrand, Flemming; Corydon, Thomas Juhl; Gregersen, Niels; Olsen, Rikke Katrine Jentoft.
In: Pediatric Research, Vol. 88, No. 4, 10.2020, p. 556-564.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
A recurrent de novo HSPD1 variant is associated with hypomyelinating leukodystrophy. / Cömert, Cagla; Brick, Lauren; Ang, Debbie; Palmfeldt, Johan; Meaney, Brandon F; Kozenko, Mariya; Georgopoulos, Costa; Fernandez-Guerra, Paula; Bross, Peter.
In: Cold Spring Harbor molecular case studies, Vol. 6, No. 3, 06.2020.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Myalgisk encefalomyelitis eller kronisk træthedssyndrom. / Brinth, Louise; Nielsen, Henrik; Varming, Kim; Boonen, Susanne E; Ebsen, A C Gonzalez; Fernández-Guerra, Paula; Schou, Anne Sophie; Mehlsen, Jesper; Gregersen, Niels; Brandslund, Ivan; Olsen, Rikke Katrine Jentoft.
In: Ugeskrift for Laeger, Vol. 181, No. 24, V08180570, 10.06.2019.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
A Cell Model for HSP60 Deficiencies: Modeling Different Levels of Chaperonopathies Leading to Oxidative Stress and Mitochondrial Dysfunction. / Cömert, C; Fernandez-Guerra, P; Bross, P.
Protein Misfolding Diseases: Methods and Protocols. ed. / Cláudio M. Gomez. Humana Press, New York, NY : Springer, 2019. p. 225-239 (Methods in Molecular Biology).Research output: Contribution to book/anthology/report/proceeding › Book chapter › Research › peer-review
Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria. / Sahebekhtiari, N; Fernandez-Guerra, P; Nochi, Z; Carlsen, J; Bross, P; Palmfeldt, J.
In: B B A - Molecular Basis of Disease, Vol. 1865, No. 1, 01.2019, p. 126-135.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
APD-Containing Cyclolipodepsipeptides Target Mitochondrial Function in Hypoxic Cancer Cells. / Jacobsen, Kristian Mark; Villadsen, Nikolaj Lilholm; Tørring, Thomas; Nielsen, Camilla Bak; Salomón, Trine; Nielsen, Morten Muhlig; Tsakos, Michail; Sibbersen, Christian; Scavenius, Carsten; Nielsen, Rikke; Christensen, Erik Ilsø; Guerra, Paula Fernandez; Bross, Peter; Pedersen, Jakob Skou; Enghild, Jan Johannes; Johannsen, Mogens; Frøkiær, Jørgen; Overgaard, Jens; Horsman, Michael R; Busk, Morten; Poulsen, Thomas B.
In: Cell Chemical Biology, Vol. 25, No. 11, 11.2018, p. 1337-1349.e12.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Metformin Targets Brown Adipose Tissue in vivo and Reduces Oxygen Consumption in vitro. / Breining, Peter; Jensen, Jonas B; Sundelin, Elias I; Gormsen, Lars C; Jakobsen, Steen; Busk, Morten; Rolighed, Lars; Bross, Peter; Fernandez-Guerra, Paula; Markussen, Lasse K; Rasmussen, Nanna E; Hansen, Jacob B; Pedersen, Steen B; Richelsen, Bjørn; Jessen, Niels.
In: Diabetes, Obesity and Metabolism, Vol. 20, No. 9, 09.2018, p. 2264-2273.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Kronisk træthedssyndrom, en usynlig sygdom? Seahorse XF teknologi kan måle cellers evne til at producere energi under stress, og afslører dysfunktionelle energisystemer i Kronisk træthedssyndrom. / Olsen, Rikke Katrine Jentoft; Martlev , L; Fernandez-Guerra, Paula; Brinth , L.
In: Dansk Kemi, Vol. 1, No. Februar 2018 / 99, 2018, p. 20-24.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Contribution to newspaper - Feature article
Mitochondrial Spare Respiratory Capacity Is Negatively Correlated With Nuclear Reprogramming Efficiency. / Zhou, Yan; Al-Saaidi, Rasha Abdelkadhem; Guerra, Paula Fernandez; Freude, Kristine Karla; Olsen, Rikke Katrine Jentoft; Jensen, Uffe Birk; Gregersen, Niels; Hyttel, Poul; Bolund, Lars; Aagaard, Lars; Bross, Peter; Luo, Yonglun.
In: Stem Cells and Development, Vol. 26, No. 3, 02.2017, p. 166-176.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Disease-Associated Mutations in the HSPD1 Gene Encoding the Large Subunit of the Mitochondrial HSP60/HSP10 Chaperonin Complex. / Bross, Peter; Fernandez-Guerra, Paula.
In: Frontiers in Molecular Biosciences, Vol. 3, 2016, p. 49.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Effects of a Mutation in the HSPE1 Gene Encoding the Mitochondrial Co-chaperonin HSP10 and Its Potential Association with a Neurological and Developmental Disorder. / Bie, Anne S; Fernandez-Guerra, Paula; Birkler, Rune I D; Nisemblat, Shahar; Pelnena, Dita; Lu, Xinping; Deignan, Joshua L; Lee, Hane; Dorrani, Naghmeh; Corydon, Thomas J; Palmfeldt, Johan; Bivina, Liga; Azem, Abdussalam; Herman, Kristin; Bross, Peter.
In: Frontiers in Molecular Biosciences, Vol. 3, 2016, p. 65.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblasts from Patients with Inherited Disorders. / Fernandez-Guerra, Paula; Lund, Martin; Corydon, T J; Cornelius, Nanna; Gregersen, Niels; Palmfeldt, J; Bross, Peter.
In: JIMD Reports , 25.09.2015.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Selected reaction monitoring as an effective method for reliable quantification of disease-associated proteins in maple syrup urine disease. / Fernández-Guerra, Paula; Birkler, Rune I D; Merinero, Begoña; Ugarte, Magdalena; Gregersen, Niels; Rodríguez-Pombo, Pilar; Bross, Peter; Palmfeldt, Johan.
In: Molecular Genetics & Genomic Medicine, Vol. 2, No. 5, 09.2014, p. 383-92.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Cellular characterization of human dermal fibroblasts, focus on mitochondria and maple syrup urine disease : PhD dissertation. / Fernandez-Guerra, Paula.
Master of Public health, Aarhus Universitet, 2014. 214 p.Research output: Book/anthology/dissertation/report › Ph.D. thesis
PROTEOMICS AND CELLULAR OXIDATIVE STRESS STUDIES IN FIBROBLASTS FROM CLASSIC MAPLE SYRUP URINE DISEASE PATIENTS. / Fernandez Guerra, Paula; Birkler, Rune Isak Dupont; Palmfeldt, Johan; Bross, Peter.
2014. Poster session presented at Aarhus University Graduate School of Health, PhD Day 2014, Aarhus, Denmark.Research output: Contribution to conference › Poster › Communication
Research output: Non-textual form › Pictures, Video and sound recordings (digital) › Communication
Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency. / Cornelius, Nanna; Byron, Colleen; Hargreaves, Iain; Guerra, Paula Fernandez; Furdek, Andrea K; Land, John; Radford, Weston W; Frerman, Frank; Corydon, Thomas J; Gregersen, Niels; Olsen, Rikke K J.
In: Human Molecular Genetics, Vol. 22, No. 19, 01.10.2013, p. 3819-27.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Fibroblasts from Maple Syrup Urine Disease (MSUD) classic patients showed mitochondrial oxidative stress and protein damage. / Fernandez Guerra, Paula; Palmfeldt, Johan; Merinero, B.; Bross, Peter; Rodriguez-Pombo, P.
2013. Poster session presented at 12th International congress of inborn errors of metabolism, Barcelona, Spain.Research output: Contribution to conference › Poster › Communication
Evidence that fibroblasts from patients affected by medium-chain acyl-CoA dehydrogenase deficiency (MCADD) are under chronic ocidative stress. / Tonin, Anelise Miotti; Fernandez Guerra, Paula; Cornelius, Nanna; Olsen, Rikke; Wajner, M.; Gregersen, Niels.
2013. Poster session presented at 12th International congress of inborn errors of metabolism, Barcelona, Spain.Research output: Contribution to conference › Poster › Communication