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Loss of mitochondrial fatty acid β-oxidation protein short-chain Enoyl-CoA hydratase disrupts oxidative phosphorylation protein complex stability and function.
FEBS J. 2023 Jan;290(1):225-246. doi: 10.1111/febs.16595. Epub 2022 Aug 24.
FEBS J. 2023.
PMID: 35962613
Free PMC article.
Sideroflexin 4 is a complex I assembly factor that interacts with the MCIA complex and is required for the assembly of the ND2 module.
Jackson TD, Crameri JJ, Muellner-Wong L, Frazier AE, Palmer CS, Formosa LE, Hock DH, Fujihara KM, Stait T, Sharpe AJ, Thorburn DR, Ryan MT, Stroud DA, Stojanovski D.
Jackson TD, et al. Among authors: crameri jj.
Proc Natl Acad Sci U S A. 2022 Mar 29;119(13):e2115566119. doi: 10.1073/pnas.2115566119. Epub 2022 Mar 25.
Proc Natl Acad Sci U S A. 2022.
PMID: 35333655
Free PMC article.
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Stimulating Mitochondrial Biogenesis with Deoxyribonucleosides Increases Functional Capacity in ECHS1-Deficient Cells.
Burgin HJ, Crameri JJ, Stojanovski D, Sanchez MIGL, Ziemann M, McKenzie M.
Burgin HJ, et al. Among authors: crameri jj.
Int J Mol Sci. 2022 Oct 20;23(20):12610. doi: 10.3390/ijms232012610.
Int J Mol Sci. 2022.
PMID: 36293464
Free PMC article.
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Mitochondrial biology and dysfunction in secondary mitochondrial disease.
Baker MJ, Crameri JJ, Thorburn DR, Frazier AE, Stojanovski D.
Baker MJ, et al. Among authors: crameri jj.
Open Biol. 2022 Dec;12(12):220274. doi: 10.1098/rsob.220274. Epub 2022 Dec 7.
Open Biol. 2022.
PMID: 36475414
Free PMC article.
Review.
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Human Tim8a, Tim8b and Tim13 are auxiliary assembly factors of mature Complex IV.
Anderson AJ, Crameri JJ, Ang CS, Malcolm TR, Kang Y, Baker MJ, Palmer CS, Sharpe AJ, Formosa LE, Ganio K, Baker MJ, McDevitt CA, Ryan MT, Maher MJ, Stojanovski D.
Anderson AJ, et al. Among authors: crameri jj.
EMBO Rep. 2023 Aug 3;24(8):e56430. doi: 10.15252/embr.202256430. Epub 2023 Jun 5.
EMBO Rep. 2023.
PMID: 37272231
Free PMC article.
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CLPB disaggregase dysfunction impacts the functional integrity of the proteolytic SPY complex.
Baker MJ, Blau KU, Anderson AJ, Palmer CS, Fielden LF, Crameri JJ, Milenkovic D, Thorburn DR, Frazier AE, Langer T, Stojanovski D.
Baker MJ, et al. Among authors: crameri jj.
J Cell Biol. 2024 Mar 4;223(3):e202305087. doi: 10.1083/jcb.202305087. Epub 2024 Jan 25.
J Cell Biol. 2024.
PMID: 38270563
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Reduced Protein Import via TIM23 SORT Drives Disease Pathology in TIMM50-Associated Mitochondrial Disease.
Crameri JJ, Palmer CS, Stait T, Jackson TD, Lynch M, Sinclair A, Frajman LE, Compton AG, Coman D, Thorburn DR, Frazier AE, Stojanovski D.
Crameri JJ, et al.
Mol Cell Biol. 2024 Jun 3:1-19. doi: 10.1080/10985549.2024.2353652. Online ahead of print.
Mol Cell Biol. 2024.
PMID: 38828998
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