Papers

 Date Name of Paper (Click name for description and access to full paper)

 Apr, 2017Frataxin Deficiency Impairs Mitochondrial Biogenesis in Cells, Mice and Humans.

 Feb, 2017

 Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model
 Dec, 2016 Galectin-3 regulates inflammasome activation in cholestatic liver injury

 Dec, 2016

 Parkin deficiency accelerates consequences of mitochondrial DNA deletions and Parkinsonism
 Oct, 2016  

  A high-throughput screen for mitochondrial function reveals known and novel mitochondrial toxicants in a library of environmental agents
 Sep, 2016 Mice with low levels of Shc proteins display reduced glycolytic and increased gluconeogenic activities in liver

 Sep, 2016 Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutations.

 Aug, 2016 

 Neurobehavioral deficits in the KIKO mouse model of Friedreich's ataxia
 June, 2016

 Lactate and Pyruvate Are Major Sources of Energy for Stallion Sperm with Dose Effects on Mitochondrial Function, Motility, and ROS Production.
 June, 2016 p46Shc Inhibits Thiolase and Lipid Oxidation in Mitochondria.

 May, 2016

 Mitochondrial oxygen consumption is a unique indicator of stallion spermatozoal health and varies with cryopreservation media
 Apr, 2016

 Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia.
 Mar, 2016

 Frataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34.
 Dec, 2015 Mitochondrial Hspa9/Mortalin regulates erythroid differentiation via iron-sulfur cluster assembly.

 Nov, 2015 Oxidative stress in inherited mitochondrial diseases

 April, 2015    
 Key Glycolytic Enzyme Activities of Skeletal Muscle Are Decreased under Fed and Fasted States in Mice with Knocked Down Levels of Shc Proteins

 Feb, 2015    
 Dysregulation of glutamine transporter SNAT1 in Rett syndrome microglia: a mechanism for mitochondrial dysfunction and neurotoxicity

 Feb, 2015    
 Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse

 Nov, 2014    
 Mitochondrial complex I defects increase ubiquitin in substantia nigra

 Sept, 2014 Shc depletion stimulates brown fat activity in-vivo and in-vitro
 Aug, 2014  Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.
 Aug, 2014 Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia.

 July, 2014 High-throughput screening of FDA-approved drugs using oxygen biosensor plates reveals secondary mitofunctional effects.

 Mar, 2013 Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model.

 Feb, 2013 

 Effects of alkyl side chain modification of coenzyme Q10 on mitochondrial respiratory chain function and cytoprotection.
 Dec, 2012 Development of an HTS assay for EPHX2 phosphatase activity and screening of nontargeted libraries.

 Oct, 2012 OPA1 Mutation and Late-Onset Cardiomyopathy: Mitochondrial Dysfunction and mtDNA Instability.

 Sept, 2012 Mutant Twinkle increases dopaminergic neurodegeneration, mtDNA deletions and modulates Parkin expression. 

 June, 2012 Shc proteins influence the activities of enzymes involved in fatty acid oxidation and ketogenesis. 

 May, 2012 Hepatic Src homology phosphatase 2 regulates energy balance in mice.

 Dec, 2011 HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis.

 Dec, 2011 The p66Shc knocked out mice are short lived under natural condition.

 Nov, 2010 The Shc locus regulates insulin signaling and adiposity in mammals.

 Aug, 2010 Synthesis and characterization of mitoQ and idebenone analogues as mediators of oxygen consumption in mitochondria.

 Dec, 2009 Oligodendroglial differentiation induces mitochondrial genes and inhibition of mitochondrial function represses oligodendroglial differentiation.

 Nov, 2009 Decreased superoxide production in macrophages of long-lived p66Shc knock-out mice.

 Oct, 2009 Pyrroloquinoline quinone stimulates mitochondrial biogenesis through cAMP response element-binding protein phosphorylation and increased PGC-1alpha expression.

 Aug, 2009 Frataxin deficiency induces Schwann cell inflammation and death.

 Feb, 2009 Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia.

 Oct, 2008 Biosensor plates detect mitochondrial physiological regulators and mutations in vivo.

 Sept, 2008 Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.

 July, 2007 Mitochondrial DNA deletions induce the adenosine monophosphate-activated protein kinase energy stress pathway and result in decreased secretion of some proteins.

 July, 2007 Mitochondrial DNA deletions and chloramphenicol treatment stimulate the autophagic transcript ATG12.

 Apr, 2007 Hemin rescues adrenodoxin, heme a and cytochrome oxidase activity in frataxin-deficient oligodendroglioma cells.

 Mar, 2007 Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones.

 Oct, 2006 Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts.

 Sept, 2006 Mitochondrial DNA deletions inhibit proteasomal activity and stimulate an autophagic transcript.

 May, 2006 Mitochondrial disease activates transcripts of the unfolded protein response and cell cycle and inhibits vesicular secretion and oligodendrocyte-specific transcripts.

 Mar, 2006 Frataxin, iron-sulfur clusters, heme, ROS, and aging.

 Feb, 2006 A low dose of ethidium bromide leads to an increase of total mitochondrial DNA while higher concentrations induce the mtDNA 4997 deletion in a human neuronal cell line
 Oct, 2005 Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells.

 Feb, 2005 Isolation of transcriptomal changes attributable to LHON mutations and the cybridization process.

 July, 2003 Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells.

 Nov, 2002 High-throughput measurement of mitochondrial membrane potential in a neural cell line using a fluorescence plate reader.

 Sept, 2002 A neutral theory predicts multigenic aging and increased concentrations of deleterious mutations on the mitochondrial and Y chromosomes.

 Apr, 2002 Fixation of deleterious alleles, evolution and human aging.

 Apr, 2002 Reproducible quantitative PCR of mitochondrial and nuclear DNA copy number using the LightCycler.

 Mar, 2002 Quantification of human mitochondrial DNA in a real time PCR.

 Feb, 2002 Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells.

 Dec, 2001 Cells bearing mutations causing Leber's hereditary optic neuropathy are sensitized to Fas-Induced apoptosis.

 Sep, 2001 Frataxin expression rescues mitochondrial dysfunctions in FRDA cells.

 Aug, 2001 Cellular characterization of leukotoxin diol-induced mitochondrial dysfunction.

 May, 2001 Genetic basis for susceptibility to noise-induced hearing loss in mice.

 Dec, 2000 Mitochondrial defects and hearing loss.

 Oct, 2000 Bcl-2 does not inhibit the permeability transition pore in mouse liver mitochondria.

 Fall, 2000 Sensitivity of FRDA lymphoblasts to salts of transition metal ions.

 Nov, 1999 Frequencies of hprt(-) mutations and bcl-2 translocations in circulating human lymphocytes are correlated with United Kingdom sunlight records.

 Mar, 1999

  The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.

 Feb, 1999

  Mitochondria in organismal aging and degeneration.
  (FULL LIST AVAILABLE ON PUBMED UNDER CORTOPASSI G)

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