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Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model.
Tegelberg S, Tomašić N, Kallijärvi J, Purhonen J, Elmér E, Lindberg E, Nord DG, Soller M, Lesko N, Wedell A, Bruhn H, Freyer C, Stranneheim H, Wibom R, Nennesmo I, Wredenberg A, Eklund EA, Fellman V. Tegelberg S, et al. Among authors: purhonen j. Orphanet J Rare Dis. 2017 Apr 20;12(1):73. doi: 10.1186/s13023-017-0624-2. Orphanet J Rare Dis. 2017. PMID: 28427446 Free PMC article.
NAD+ repletion produces no therapeutic effect in mice with respiratory chain complex III deficiency and chronic energy deprivation.
Purhonen J, Rajendran J, Tegelberg S, Smolander OP, Pirinen E, Kallijärvi J, Fellman V. Purhonen J, et al. FASEB J. 2018 May 21:fj201800090R. doi: 10.1096/fj.201800090R. Online ahead of print. FASEB J. 2018. PMID: 29782205
Our study highlights that the knowledge of the underlying complex metabolic alterations is critical when designing therapies for mitochondrial dysfunction.-Purhonen, J., Rajendran, J., Tegelberg, S., Smolander, O.-P., Pirinen, E., Kallijarvi, J., Fellm …
Our study highlights that the knowledge of the underlying complex metabolic alterations is critical when designing therapies for mitochondri …
Alternative oxidase-mediated respiration prevents lethal mitochondrial cardiomyopathy.
Rajendran J, Purhonen J, Tegelberg S, Smolander OP, Mörgelin M, Rozman J, Gailus-Durner V, Fuchs H, Hrabe de Angelis M, Auvinen P, Mervaala E, Jacobs HT, Szibor M, Fellman V, Kallijärvi J. Rajendran J, et al. Among authors: purhonen j. EMBO Mol Med. 2019 Jan;11(1):e9456. doi: 10.15252/emmm.201809456. EMBO Mol Med. 2019. PMID: 30530468 Free PMC article.
Altered acylcarnitine metabolism and inflexible mitochondrial fuel utilization characterize the loss of neonatal myocardial regeneration capacity.
Kankuri E, Finckenberg P, Leinonen J, Tarkia M, Björk S, Purhonen J, Kallijärvi J, Kankainen M, Soliymani R, Lalowski M, Mervaala E. Kankuri E, et al. Among authors: purhonen j. Exp Mol Med. 2023 Apr;55(4):806-817. doi: 10.1038/s12276-023-00967-5. Epub 2023 Apr 3. Exp Mol Med. 2023. PMID: 37009793 Free PMC article.
19 results