Erika Fernandez-Vizarra

Erika Fernandez-Vizarra

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Erika Fernandez-Vizarra

Erika Fernandez-Vizarra

Publications by authors named "Erika Fernandez-Vizarra"

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Mitochondrial complex III Rieske Fe-S protein processing and assembly.

Cell Cycle 2018 10;17(6):681-687. Epub 2018 Apr 10.

a MRC-Mitochondrial Biology Unit , University of Cambridge , Hills Road, CB2 0XY , Cambridge , UK.

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http://dx.doi.org/10.1080/15384101.2017.1417707DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5969560PMC
September 2019

Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes.

Essays Biochem 2018 07 20;62(3):255-270. Epub 2018 Jul 20.

MRC-Mitochondrial Biology Unit, University of Cambridge, Hills Road, Cambridge CB2 0XY, U.K.

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http://dx.doi.org/10.1042/EBC20170098DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056720PMC
July 2018

Cavitating Leukoencephalopathy With Posterior Predominance Caused by a Deletion in the APOPT1 Gene in an Indian Boy.

J Child Neurol 2018 05 26;33(6):428-431. Epub 2018 Mar 26.

4 Department of Pathology, G.B. Pant Hospital, New Delhi, India.

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http://dx.doi.org/10.1177/0883073818760875DOI Listing
May 2018

TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.

Mol Cell 2017 Jul 29;67(1):96-105.e4. Epub 2017 Jun 29.

MRC Mitochondrial Biology Unit, University of Cambridge, Wellcome Trust/MRC Building Hills Road, Cambridge CB2 0XY, UK. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2017.06.001DOI Listing
July 2017

COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation.

Cell Rep 2016 08 18;16(9):2387-98. Epub 2016 Aug 18.

Instituto de Investigación, Hospital Universitario 12 de Octubre (i+12), Madrid 28041, Spain; Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), U723, Madrid 28029, Spain. Electronic address:

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http://dx.doi.org/10.1016/j.celrep.2016.07.081DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5007171PMC
August 2016

Nuclear gene mutations as the cause of mitochondrial complex III deficiency.

Front Genet 2015 9;6:134. Epub 2015 Apr 9.

Mitochondrial Biology Unit, Medical Research Council Cambridge, UK.

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http://dx.doi.org/10.3389/fgene.2015.00134DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4391031PMC
April 2015

LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells.

Biochim Biophys Acta 2013 Mar 17;1827(3):285-93. Epub 2012 Nov 17.

Unidad de Investigación Traslacional, Hospital Universitario Miguel Servet, Zaragoza, Spain.

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http://dx.doi.org/10.1016/j.bbabio.2012.11.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3570683PMC
March 2013

Proteomics and gene expression analyses of mitochondria from squalene-treated apoE-deficient mice identify short-chain specific acyl-CoA dehydrogenase changes associated with fatty liver amelioration.

J Proteomics 2012 May 3;75(9):2563-75. Epub 2012 Mar 3.

Departamento Bioquímica y Biología Molecular y Celular, Instituto de Investigación Sanitaria de Aragón, Universidad de Zaragoza, Spain.

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http://dx.doi.org/10.1016/j.jprot.2012.02.025DOI Listing
May 2012

Partial tandem duplication of mtDNA-tRNA(Phe) impairs mtDNA translation in late-onset mitochondrial myopathy.

Neuromuscul Disord 2012 Jan 9;22(1):50-5. Epub 2012 Jan 9.

Unit of Molecular Neurogenetics, The Foundation "Carlo Besta" Institute of Neurology-IRCCS, Milan, Italy.

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http://linkinghub.elsevier.com/retrieve/pii/S096089661101313
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http://dx.doi.org/10.1016/j.nmd.2011.07.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3334271PMC
January 2012

Nonsense mutation in pseudouridylate synthase 1 (PUS1) in two brothers affected by myopathy, lactic acidosis and sideroblastic anaemia (MLASA).

BMJ Case Rep 2009 9;2009. Epub 2009 Jun 9.

Division of Molecular Neurogenetics, Pierfranco and Luisa Mariani Center for the Study of Mitochondrial Disorders of Infancy and Childhood, National Institute of Neurology "C. Besta", Milan, Italy.

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http://search.proquest.com/openview/99101163f18bed50b4cf3e7b
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http://casereports.bmj.com/cgi/doi/10.1136/bcr.05.2009.1889
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http://dx.doi.org/10.1136/bcr.05.2009.1889DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3030164PMC
November 2011

Tissue-specific differences in mitochondrial activity and biogenesis.

Mitochondrion 2011 Jan 7;11(1):207-13. Epub 2010 Oct 7.

Departamento de Bioquímica y Biología Molecular y Celular, Universidad de Zaragoza, Pedro Cerbuna, 12. 50009 Zaragoza, Spain.

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http://dx.doi.org/10.1016/j.mito.2010.09.011DOI Listing
January 2011

Isolation of mitochondria for biogenetical studies: An update.

Mitochondrion 2010 Apr 23;10(3):253-62. Epub 2009 Dec 23.

Departamento de Bioquímica y Biología Molecular y Celular, Universidad de Zaragoza, Pedro Cerbuna 12, 50009 Zaragoza, Spain.

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http://dx.doi.org/10.1016/j.mito.2009.12.148DOI Listing
April 2010

How do human cells react to the absence of mitochondrial DNA?

PLoS One 2009 May 28;4(5):e5713. Epub 2009 May 28.

Unit of Molecular Neurogenetics-Pierfranco and Luisa Mariani Center for the study of Mitochondrial Disorders in Children, IRCCS Foundation Neurological Institute C. Besta, Milan, Italy.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0005713PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2683933PMC
May 2009

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

Biochim Biophys Acta 2009 Jan 21;1793(1):200-11. Epub 2008 Jun 21.

Department of Molecular Neurogenetics, IRCCS Foundation Neurological Institute C. Besta, 20126 Milan, Italy.

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http://dx.doi.org/10.1016/j.bbamcr.2008.05.028DOI Listing
January 2009

Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice.

Hum Mol Genet 2009 Jan 24;18(1):12-26. Epub 2008 Sep 24.

Unit of Molecular Neurogenetics - Pierfranco and Luisa Mariani Center for the Study of Mitochondrial Disorders in Children, IRCCS Foundation Neurological Institute C. Besta, Milan, Italy.

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http://dx.doi.org/10.1093/hmg/ddn309DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2644642PMC
January 2009

FASTKD2 nonsense mutation in an infantile mitochondrial encephalomyopathy associated with cytochrome c oxidase deficiency.

Am J Hum Genet 2008 Sep 4;83(3):415-23. Epub 2008 Sep 4.

Division of Molecular Neurogenetics, Foundation IRCCS Neurological Institute C. Besta, 20126 Milan, Italy.

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http://dx.doi.org/10.1016/j.ajhg.2008.08.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2556431PMC
September 2008

Mitochondrial gene expression is regulated at multiple levels and differentially in the heart and liver by thyroid hormones.

Curr Genet 2008 Jul 15;54(1):13-22. Epub 2008 May 15.

Departamento de Bioquímica y Biología Molecular y Celular, Universidad de Zaragoza, Miguel Servet 177, Zaragoza, Spain.

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http://dx.doi.org/10.1007/s00294-008-0194-xDOI Listing
July 2008

Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy.

Hum Mol Genet 2007 May 2;16(10):1241-52. Epub 2007 Apr 2.

Department of Molecular Neurogenetics, , Foundation IRCCS Neurological Institute C. Besta, Milano, Italy.

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http://academic.oup.com/hmg/article/16/10/1241/628176/Impair
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http://dx.doi.org/10.1093/hmg/ddm072DOI Listing
May 2007

Nonsense mutation in pseudouridylate synthase 1 (PUS1) in two brothers affected by myopathy, lactic acidosis and sideroblastic anaemia (MLASA).

J Med Genet 2007 Mar 20;44(3):173-80. Epub 2006 Oct 20.

Division of Molecular Neurogenetics, Pierfranco and Luisa Mariani Center for the Study of Mitochondrial Disorders of Infancy and Childhood, National Institute of Neurology C. Besta, Milan, Italy.

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http://dx.doi.org/10.1136/jmg.2006.045252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2598032PMC
March 2007

Infantile encephalopathy and defective mitochondrial DNA translation in patients with mutations of mitochondrial elongation factors EFG1 and EFTu.

Am J Hum Genet 2007 Jan 15;80(1):44-58. Epub 2006 Nov 15.

Pierfranco and Luisa Mariani Center for Research on Children's Mitochondrial Disorders, Division of Molecular Neurogenetics, National Neurological Institute "Carlo Besta," Milano, Italy.

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http://dx.doi.org/10.1086/510559DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1785320PMC
January 2007

MPV17 encodes an inner mitochondrial membrane protein and is mutated in infantile hepatic mitochondrial DNA depletion.

Nat Genet 2006 May 2;38(5):570-5. Epub 2006 Apr 2.

Unit of Molecular Neurogenetics, Pierfranco and Luisa Mariani Center for the Study of Children's Mitochondrial Disorders, National Neurological Institute C. Besta, Milan 20126, Italy.

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http://dx.doi.org/10.1038/ng1765DOI Listing
May 2006

In vitro transcription termination activity of the Drosophila mitochondrial DNA-binding protein DmTTF.

Biochem Biophys Res Commun 2005 May;331(1):357-62

Dipartimento di Biochimica e Biologia Molecolare, Università degli Studi di Bari, Via Orabona, 4, 70125 Bari, Italy.

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http://dx.doi.org/10.1016/j.bbrc.2005.03.173DOI Listing
May 2005

Redox activation of mitochondrial intermembrane space Cu,Zn-superoxide dismutase.

Biochem J 2005 Apr;387(Pt 1):203-9

Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza. 50009-Zaragoza, Spain.

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http://dx.doi.org/10.1042/BJ20041683DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1134948PMC
April 2005

Isolation of biogenetically competent mitochondria from mammalian tissues and cultured cells.

Methods 2002 Apr;26(4):292-7

Departamento de Bioquímica y Biología Molecular y Celular, Universidad de Zaragoza, Miguel Servet, 177, Zaragoza 50013, Spain.

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http://dx.doi.org/10.1016/S1046-2023(02)00034-8DOI Listing
April 2002