Similar articles for PMID: 20200222

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1

LRPPRC and SLIRP interact in a ribonucleoprotein complex that regulates posttranscriptional gene expression in mitochondria.

Sasarman F, Brunel-Guitton C, Antonicka H, Wai T, Shoubridge EA; LSFC Consortium. Sasarman F, et al. Mol Biol Cell. 2010 Apr 15;21(8):1315-23. doi: 10.1091/mbc.e10-01-0047. Epub 2010 Mar 3. Mol Biol Cell. 2010. PMID: 20200222 Free PMC article.

2

Tissue-specific responses to the LRPPRC founder mutation in French Canadian Leigh Syndrome.

Sasarman F, Nishimura T, Antonicka H, Weraarpachai W, Shoubridge EA; LSFC Consortium. Sasarman F, et al. Hum Mol Genet. 2015 Jan 15;24(2):480-91. doi: 10.1093/hmg/ddu468. Epub 2014 Sep 11. Hum Mol Genet. 2015. PMID: 25214534 Free PMC article.

3

mTORC1 is required for expression of LRPPRC and cytochrome-c oxidase but not HIF-1α in Leigh syndrome French Canadian type patient fibroblasts.

Mukaneza Y, Cohen A, Rivard MÈ, Tardif J, Deschênes S, Ruiz M; LSFC Consortium; Laprise C, Des Rosiers C, Coderre L. Mukaneza Y, et al. Am J Physiol Cell Physiol. 2019 Jul 1;317(1):C58-C67. doi: 10.1152/ajpcell.00160.2017. Epub 2019 Apr 17. Am J Physiol Cell Physiol. 2019. PMID: 30995105 Free PMC article.

4

The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA.

Xu F, Morin C, Mitchell G, Ackerley C, Robinson BH. Xu F, et al. Biochem J. 2004 Aug 15;382(Pt 1):331-6. doi: 10.1042/BJ20040469. Biochem J. 2004. PMID: 15139850 Free PMC article.

5

LRPPRC mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.

Oláhová M, Hardy SA, Hall J, Yarham JW, Haack TB, Wilson WC, Alston CL, He L, Aznauryan E, Brown RM, Brown GK, Morris AA, Mundy H, Broomfield A, Barbosa IA, Simpson MA, Deshpande C, Moeslinger D, Koch J, Stettner GM, Bonnen PE, Prokisch H, Lightowlers RN, McFarland R, Chrzanowska-Lightowlers ZM, Taylor RW. Oláhová M, et al. Brain. 2015 Dec;138(Pt 12):3503-19. doi: 10.1093/brain/awv291. Epub 2015 Oct 27. Brain. 2015. PMID: 26510951 Free PMC article.

6

Loss of hepatic LRPPRC alters mitochondrial bioenergetics, regulation of permeability transition and trans-membrane ROS diffusion.

Cuillerier A, Honarmand S, Cadete VJJ, Ruiz M, Forest A, Deschênes S, Beauchamp C; LSFC Consortium; Charron G, Rioux JD, Des Rosiers C, Shoubridge EA, Burelle Y. Cuillerier A, et al. Hum Mol Genet. 2017 Aug 15;26(16):3186-3201. doi: 10.1093/hmg/ddx202. Hum Mol Genet. 2017. PMID: 28575497 Free PMC article.

7

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs.

Ruzzenente B, Metodiev MD, Wredenberg A, Bratic A, Park CB, Cámara Y, Milenkovic D, Zickermann V, Wibom R, Hultenby K, Erdjument-Bromage H, Tempst P, Brandt U, Stewart JB, Gustafsson CM, Larsson NG. Ruzzenente B, et al. EMBO J. 2012 Jan 18;31(2):443-56. doi: 10.1038/emboj.2011.392. Epub 2011 Nov 1. EMBO J. 2012. PMID: 22045337 Free PMC article.

8

Expression signature of the Leigh syndrome French-Canadian type.

Bchetnia M, Tardif J, Morin C, Laprise C. Bchetnia M, et al. Mol Genet Metab Rep. 2022 Feb 5;30:100847. doi: 10.1016/j.ymgmr.2022.100847. eCollection 2022 Mar. Mol Genet Metab Rep. 2022. PMID: 35242578 Free PMC article.

9

SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation.

Lagouge M, Mourier A, Lee HJ, Spåhr H, Wai T, Kukat C, Silva Ramos E, Motori E, Busch JD, Siira S; German Mouse Clinic Consortium; Kremmer E, Filipovska A, Larsson NG. Lagouge M, et al. PLoS Genet. 2015 Aug 6;11(8):e1005423. doi: 10.1371/journal.pgen.1005423. eCollection 2015 Aug. PLoS Genet. 2015. PMID: 26247782 Free PMC article.

10

Mitochondrial vulnerability and increased susceptibility to nutrient-induced cytotoxicity in fibroblasts from leigh syndrome French canadian patients.

Burelle Y, Bemeur C, Rivard ME, Thompson Legault J, Boucher G; LSFC Consortium; Morin C, Coderre L, Des Rosiers C. Burelle Y, et al. PLoS One. 2015 Apr 2;10(3):e0120767. doi: 10.1371/journal.pone.0120767. eCollection 2015. PLoS One. 2015. PMID: 25835550 Free PMC article.

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