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Items: 1 to 20 of 105

1.

Mouse models of oxidative phosphorylation defects: powerful tools to study the pathobiology of mitochondrial diseases.

Torraco A, Diaz F, Vempati UD, Moraes CT.

Biochim Biophys Acta. 2009 Jan;1793(1):171-80. doi: 10.1016/j.bbamcr.2008.06.003. Epub 2008 Jun 13. Review.

2.

Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects.

Fernández-Vizarra E, Tiranti V, Zeviani M.

Biochim Biophys Acta. 2009 Jan;1793(1):200-11. doi: 10.1016/j.bbamcr.2008.05.028. Epub 2008 Jun 21. Review.

3.

Mouse models of oxidative phosphorylation dysfunction and disease.

Vempati UD, Torraco A, Moraes CT.

Methods. 2008 Dec;46(4):241-7. doi: 10.1016/j.ymeth.2008.09.008. Epub 2008 Oct 10.

4.

Mitochondrial Diseases Part I: mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors.

Torraco A, Peralta S, Iommarini L, Diaz F.

Mitochondrion. 2015 Mar;21:76-91. doi: 10.1016/j.mito.2015.01.009. Epub 2015 Feb 4. Review.

5.

The striatum is highly susceptible to mitochondrial oxidative phosphorylation dysfunctions.

Pickrell AM, Fukui H, Wang X, Pinto M, Moraes CT.

J Neurosci. 2011 Jul 6;31(27):9895-904. doi: 10.1523/JNEUROSCI.6223-10.2011.

6.

Mitochondrial diseases: Drosophila melanogaster as a model to evaluate potential therapeutics.

Foriel S, Willems P, Smeitink J, Schenck A, Beyrath J.

Int J Biochem Cell Biol. 2015 Jun;63:60-5. doi: 10.1016/j.biocel.2015.01.024. Epub 2015 Feb 7. Review.

PMID:
25666557
7.

Mouse models of mitochondrial complex I dysfunction.

Irwin MH, Parameshwaran K, Pinkert CA.

Int J Biochem Cell Biol. 2013 Jan;45(1):34-40. doi: 10.1016/j.biocel.2012.08.009. Epub 2012 Aug 10. Review.

8.

Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency.

Koene S, Willems PH, Roestenberg P, Koopman WJ, Smeitink JA.

J Inherit Metab Dis. 2011 Apr;34(2):293-307. doi: 10.1007/s10545-009-9005-x. Epub 2010 Jan 27. Review.

PMID:
20107904
9.

Mechanisms of mitochondrial diseases.

Ylikallio E, Suomalainen A.

Ann Med. 2012 Feb;44(1):41-59. doi: 10.3109/07853890.2011.598547. Epub 2011 Aug 2.

PMID:
21806499
10.

Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function.

Iommarini L, Peralta S, Torraco A, Diaz F.

Mitochondrion. 2015 May;22:96-118. doi: 10.1016/j.mito.2015.01.008. Epub 2015 Jan 29. Review.

12.

Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficiencies.

Smits P, Smeitink J, van den Heuvel L.

J Biomed Biotechnol. 2010;2010:737385. doi: 10.1155/2010/737385. Epub 2010 Apr 13. Review.

13.

The expanding spectrum of nuclear gene mutations in mitochondrial disorders.

Zeviani M.

Semin Cell Dev Biol. 2001 Dec;12(6):407-16. Review.

PMID:
11735375
14.

Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies.

Peralta S, Torraco A, Iommarini L, Diaz F.

Mitochondrion. 2015 Jul;23:71-80. doi: 10.1016/j.mito.2015.01.007. Epub 2015 Jan 29. Review.

16.

Genetic control of oxidative phosphorylation and experimental models of defects.

Trounce I.

Hum Reprod. 2000 Jul;15 Suppl 2:18-27. Review.

PMID:
11041510
17.

Neonatal cardiomyopathies and metabolic crises due to oxidative phosphorylation defects.

Schiff M, Ogier de Baulny H, Lombès A.

Semin Fetal Neonatal Med. 2011 Aug;16(4):216-21. doi: 10.1016/j.siny.2011.04.002. Epub 2011 May 24. Review.

PMID:
21606011
18.

Mitochondrial biogenesis: pharmacological approaches.

Valero T.

Curr Pharm Des. 2014;20(35):5507-9.

PMID:
24606795
19.

Mitochondrial disorders caused by mutations in respiratory chain assembly factors.

Diaz F, Kotarsky H, Fellman V, Moraes CT.

Semin Fetal Neonatal Med. 2011 Aug;16(4):197-204. doi: 10.1016/j.siny.2011.05.004. Epub 2011 Jun 15. Review.

20.

Human mitochondrial DNA: roles of inherited and somatic mutations.

Schon EA, DiMauro S, Hirano M.

Nat Rev Genet. 2012 Dec;13(12):878-90. doi: 10.1038/nrg3275. Review.

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