228 related articles for article (PubMed ID: 31341105)
1. Lipidomics unveils lipid dyshomeostasis and low circulating plasmalogens as biomarkers in a monogenic mitochondrial disorder.
Ruiz M; Cuillerier A; Daneault C; Deschênes S; Frayne IR; Bouchard B; Forest A; Legault JT; ; Vaz FM; Rioux JD; Burelle Y; Des Rosiers C
JCI Insight; 2019 Jul; 4(14):. PubMed ID: 31341105
[TBL] [Abstract][Full Text] [Related]
2. 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; ; Charron G; Rioux JD; Des Rosiers C; Shoubridge EA; Burelle Y
Hum Mol Genet; 2017 Aug; 26(16):3186-3201. PubMed ID: 28575497
[TBL] [Abstract][Full Text] [Related]
3. A Metabolic Signature of Mitochondrial Dysfunction Revealed through a Monogenic Form of Leigh Syndrome.
Thompson Legault J; Strittmatter L; Tardif J; Sharma R; Tremblay-Vaillancourt V; Aubut C; Boucher G; Clish CB; Cyr D; Daneault C; Waters PJ; ; Vachon L; Morin C; Laprise C; Rioux JD; Mootha VK; Des Rosiers C
Cell Rep; 2015 Nov; 13(5):981-9. PubMed ID: 26565911
[TBL] [Abstract][Full Text] [Related]
4. Tissue-specific responses to the LRPPRC founder mutation in French Canadian Leigh Syndrome.
Sasarman F; Nishimura T; Antonicka H; Weraarpachai W; Shoubridge EA;
Hum Mol Genet; 2015 Jan; 24(2):480-91. PubMed ID: 25214534
[TBL] [Abstract][Full Text] [Related]
5. 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; ; Morin C; Coderre L; Des Rosiers C
PLoS One; 2015; 10(3):e0120767. PubMed ID: 25835550
[TBL] [Abstract][Full Text] [Related]
6. Novel LRPPRC compound heterozygous mutation in a child with early-onset Leigh syndrome French-Canadian type: case report of an Italian patient.
Piro E; Serra G; Antona V; Giuffrè M; Giorgio E; Sirchia F; Schierz IAM; Brusco A; Corsello G
Ital J Pediatr; 2020 Sep; 46(1):140. PubMed ID: 32972427
[TBL] [Abstract][Full Text] [Related]
7. mTORC1 is required for expression of LRPPRC and cytochrome-
Mukaneza Y; Cohen A; Rivard MÈ; Tardif J; Deschênes S; Ruiz M; ; Laprise C; Des Rosiers C; Coderre L
Am J Physiol Cell Physiol; 2019 Jul; 317(1):C58-C67. PubMed ID: 30995105
[TBL] [Abstract][Full Text] [Related]
8. Alteration of Liver Peroxisomal and Mitochondrial Functionality in the NZO Mouse Model of Metabolic Syndrome.
Knebel B; Göddeke S; Hartwig S; Hörbelt T; Fahlbusch P; Al-Hasani H; Jacob S; Koellmer C; Nitzgen U; Schiller M; Lehr S; Kotzka J
Proteomics Clin Appl; 2018 Jan; 12(1):. PubMed ID: 29068532
[TBL] [Abstract][Full Text] [Related]
9. Expression signature of the Leigh syndrome French-Canadian type.
Bchetnia M; Tardif J; Morin C; Laprise C
Mol Genet Metab Rep; 2022 Mar; 30():100847. PubMed ID: 35242578
[TBL] [Abstract][Full Text] [Related]
10. Circulating acylcarnitine profile in human heart failure: a surrogate of fatty acid metabolic dysregulation in mitochondria and beyond.
Ruiz M; Labarthe F; Fortier A; Bouchard B; Thompson Legault J; Bolduc V; Rigal O; Chen J; Ducharme A; Crawford PA; Tardif JC; Des Rosiers C
Am J Physiol Heart Circ Physiol; 2017 Oct; 313(4):H768-H781. PubMed ID: 28710072
[TBL] [Abstract][Full Text] [Related]
11. Genetic therapy in a mitochondrial disease model suggests a critical role for liver dysfunction in mortality.
Sabharwal A; Wishman MD; Cervera RL; Serres MR; Anderson JL; Holmberg SR; Kar B; Treichel AJ; Ichino N; Liu W; Yang J; Ding Y; Deng Y; Lacey JM; Laxen WJ; Loken PR; Oglesbee D; Farber SA; Clark KJ; Xu X; Ekker SC
Elife; 2022 Nov; 11():. PubMed ID: 36408801
[TBL] [Abstract][Full Text] [Related]
12. 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;
Mol Biol Cell; 2010 Apr; 21(8):1315-23. PubMed ID: 20200222
[TBL] [Abstract][Full Text] [Related]
13. Plasma lipidomics as a diagnostic tool for peroxisomal disorders.
Herzog K; Pras-Raves ML; Ferdinandusse S; Vervaart MAT; Luyf ACM; van Kampen AHC; Wanders RJA; Waterham HR; Vaz FM
J Inherit Metab Dis; 2018 May; 41(3):489-498. PubMed ID: 29209936
[TBL] [Abstract][Full Text] [Related]
14. Low-concentration methylene blue maintains energy production and strongly improves survival of Leigh syndrome French Canadian skin fibroblasts.
Legault J; Larouche PL; Côté I; Bouchard L; Pichette A; Robinson BH; Morin C
J Pharm Pharm Sci; 2011; 14(3):438-49. PubMed ID: 22202226
[TBL] [Abstract][Full Text] [Related]
15. 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
EMBO J; 2012 Jan; 31(2):443-56. PubMed ID: 22045337
[TBL] [Abstract][Full Text] [Related]
16. Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient.
Violante S; Ijlst L; Te Brinke H; Koster J; Tavares de Almeida I; Wanders RJ; Ventura FV; Houten SM
Biochim Biophys Acta; 2013 Sep; 1831(9):1467-74. PubMed ID: 23850792
[TBL] [Abstract][Full Text] [Related]
17. Medium-chain dicarboxylic acylcarnitines as markers of n-3 PUFA-induced peroxisomal oxidation of fatty acids.
Fiamoncini J; Lima TM; Hirabara SM; Ecker J; Gorjão R; Romanatto T; ELolimy A; Worsch S; Laumen H; Bader B; Daniel H; Curi R
Mol Nutr Food Res; 2015 Aug; 59(8):1573-83. PubMed ID: 25913736
[TBL] [Abstract][Full Text] [Related]
18. 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
Biochem J; 2004 Aug; 382(Pt 1):331-6. PubMed ID: 15139850
[TBL] [Abstract][Full Text] [Related]
19. Peroxisomal regulation of energy homeostasis: Effect on obesity and related metabolic disorders.
Kleiboeker B; Lodhi IJ
Mol Metab; 2022 Nov; 65():101577. PubMed ID: 35988716
[TBL] [Abstract][Full Text] [Related]
20. Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4.
Violante S; Achetib N; van Roermund CWT; Hagen J; Dodatko T; Vaz FM; Waterham HR; Chen H; Baes M; Yu C; Argmann CA; Houten SM
FASEB J; 2019 Mar; 33(3):4355-4364. PubMed ID: 30540494
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]