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Items: 9


Functional Characterization of Three Concomitant MtDNA LHON Mutations Shows No Synergistic Effect on Mitochondrial Activity.

Cruz-Bermúdez A, Vicente-Blanco RJ, Hernández-Sierra R, Montero M, Alvarez J, González Manrique M, Blázquez A, Martín MA, Ayuso C, Garesse R, Fernández-Moreno MA.

PLoS One. 2016 Jan 19;11(1):e0146816. doi: 10.1371/journal.pone.0146816. eCollection 2016.


Glutamyl-tRNAGln amidotransferase is essential for mammalian mitochondrial translation in vivo.

Echevarría L, Clemente P, Hernández-Sierra R, Gallardo ME, Fernández-Moreno MA, Garesse R.

Biochem J. 2014 May 15;460(1):91-101. doi: 10.1042/BJ20131107.


Muscle-specific overexpression of the catalytic subunit of DNA polymerase γ induces pupal lethality in Drosophila melanogaster.

Martínez-Azorín F, Calleja M, Hernández-Sierra R, Farr CL, Kaguni LS, Garesse R.

Arch Insect Biochem Physiol. 2013 Jul;83(3):127-37. doi: 10.1002/arch.21101. Epub 2013 May 31.


Evaluation of mitochondrial function and metabolic reprogramming during tumor progression in a cell model of skin carcinogenesis.

Vallejo CG, Cruz-Bermúdez A, Clemente P, Hernández-Sierra R, Garesse R, Quintanilla M.

Biochimie. 2013 Jun;95(6):1171-6. doi: 10.1016/j.biochi.2013.01.001. Epub 2013 Jan 22.


Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects.

Sanchez-Martinez A, Calleja M, Peralta S, Matsushima Y, Hernandez-Sierra R, Whitworth AJ, Kaguni LS, Garesse R.

PLoS One. 2012;7(8):e43954. doi: 10.1371/journal.pone.0043954. Epub 2012 Aug 28.


Coiled coil domain-containing protein 56 (CCDC56) is a novel mitochondrial protein essential for cytochrome c oxidase function.

Peralta S, Clemente P, Sánchez-Martínez A, Calleja M, Hernández-Sierra R, Matsushima Y, Adán C, Ugalde C, Fernández-Moreno MÁ, Kaguni LS, Garesse R.

J Biol Chem. 2012 Jul 13;287(29):24174-85. doi: 10.1074/jbc.M112.343764. Epub 2012 May 18.


Mitochondrial transcription factor B2 is essential for metabolic function in Drosophila melanogaster development.

Adán C, Matsushima Y, Hernández-Sierra R, Marco-Ferreres R, Fernández-Moreno MA, González-Vioque E, Calleja M, Aragón JJ, Kaguni LS, Garesse R.

J Biol Chem. 2008 May 2;283(18):12333-42. doi: 10.1074/jbc.M801342200. Epub 2008 Feb 28.


Over-expression of the catalytic core of mitochondrial DNA (mtDNA) polymerase in the nervous system of Drosophila melanogaster reduces median life span by inducing mtDNA depletion.

Martínez-Azorín F, Calleja M, Hernández-Sierra R, Farr CL, Kaguni LS, Garesse R.

J Neurochem. 2008 Apr;105(1):165-76. Epub 2007 Nov 12.


Modeling human mitochondrial diseases in flies.

Sánchez-Martínez A, Luo N, Clemente P, Adán C, Hernández-Sierra R, Ochoa P, Fernández-Moreno MA, Kaguni LS, Garesse R.

Biochim Biophys Acta. 2006 Sep-Oct;1757(9-10):1190-8. Epub 2006 May 13. Review.

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