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Papers

Sept, 2014


Name of Paper (Click name for description and full access to paper)
Frataxin Deficiency Impairs Mitochondrial Biogenesis in Cells, Mice and Humans
Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model
Galectin-3 regulates inflammasome activation in cholestatic liver injury
Parkin deficiency accelerates consequences of mitochondrial DNA deletions and Parkinsonism
A high-throughput screen for mitochondrial function reveals known and novel mitochondrial toxicants in a library of environmental agents
Mice with low levels of Shc proteins display reduced glycolytic and increased gluconeogenic activities in liver
Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutations
Neurobehavioral deficits in the KIKO mouse model of Friedreich's ataxia
Lactate and Pyruvate Are Major Sources of Energy for Stallion Sperm with Dose Effects on Mitochondrial Function, Motility, and ROS Production
p46Shc Inhibits Thiolase and Lipid Oxidation in Mitochondria
Mitochondrial oxygen consumption is a unique indicator of stallion spermatozoal health and varies with cryopreservation media
Lymphoblast Oxidative Stress Genes as Potential Biomarkers of Disease Severity and Drug Effect in Friedreich's Ataxia
Frataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34
Mitochondrial Hspa9/Mortalin regulates erythroid differentiation via iron-sulfur cluster assembly
Oxidative stress in inherited mitochondrial diseases
Key Glycolytic Enzyme Activities of Skeletal Muscle Are Decreased under Fed and Fasted States in Mice with Knocked Down Levels of Shc Proteins
Dysregulation of glutamine transporter SNAT1 in Rett syndrome microglia: a mechanism for mitochondrial dysfunction and neurotoxicity
Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse
Mitochondrial complex I defects increase ubiquitin in substantia nigra
Shc depletion stimulates brown fat activity in-vivo and in-vitro
Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia
Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia
High-throughput screening of FDA-approved drugs using oxygen biosensor plates reveals secondary mitofunctional effects
Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse ...
Effects of alkyl side chain modification of coenzyme Q10 on mitochondrial respiratory chain function and cytoprotection
Development of an HTS assay for EPHX2 phosphatase activity and screening of nontargeted libraries
OPA1 Mutation and Late-Onset Cardiomyopathy: Mitochondrial Dysfunction and mtDNA Instability
Mutant Twinkle increases dopaminergic neurodegeneration, mtDNA deletions and modulates Parkin expression
Shc proteins influence the activities of enzymes involved in fatty acid oxidation and ketogenesis
Hepatic Src homology phosphatase 2 regulates energy balance in mice
HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis
The p66Shc knocked out mice are short lived under natural condition
The Shc locus regulates insulin signaling and adiposity in mammals
Synthesis and characterization of mitoQ and idebenone analogues as mediators of oxygen consumption in mitochondria
Oligodendroglial differentiation induces mitochondrial genes and inhibition of mitochondrial function represses oligodendroglial differentiation
Decreased superoxide production in macrophages of long-lived p66Shc knock-out mice
Pyrroloquinoline quinone stimulates mitochondrial biogenesis through cAMP response element-binding protein phosphorylation and increased ...
Frataxin deficiency induces Schwann cell inflammation and death
Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia
Biosensor plates detect mitochondrial physiological regulators and mutations in vivo
Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation
Mitochondrial DNA deletions induce the adenosine monophosphate-activated protein kinase energy stress pathway and result in decreased ...
Mitochondrial DNA deletions and chloramphenicol treatment stimulate the autophagic transcript ATG12
Hemin rescues adrenodoxin, heme a and cytochrome oxidase activity in frataxin-deficient oligodendroglioma cells
Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones
Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts
Mitochondrial DNA deletions inhibit proteasomal activity and stimulate an autophagic transcript
Mitochondrial disease activates transcripts of the unfolded protein response and cell cycle and inhibits vesicular secretion and oligodendrocyte- ...
​Frataxin, iron-sulfur clusters, heme, ROS, and aging
A low dose of ethidium bromide leads to an increase of total mitochondrial DNA while higher concentrations induce the mtDNA 4997 deletion in a ...
Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells
Isolation of transcriptomal changes attributable to LHON mutations and the cybridization process
Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells
High-throughput measurement of mitochondrial membrane potential in a neural cell line using a fluorescence plate reader
A neutral theory predicts multigenic aging and increased concentrations of deleterious mutations on the mitochondrial and Y chromosomes
Fixation of deleterious alleles, evolution and human aging
Reproducible quantitative PCR of mitochondrial and nuclear DNA copy number using the LightCycler
Quantification of human mitochondrial DNA in a real time PCR
Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells
Cells bearing mutations causing Leber's hereditary optic neuropathy are sensitized to Fas-Induced apoptosis
Frataxin expression rescues mitochondrial dysfunctions in FRDA cells
Cellular characterization of leukotoxin diol-induced mitochondrial dysfunction
Genetic basis for susceptibility to noise-induced hearing loss in mice
Mitochondrial defects and hearing loss
Bcl-2 does not inhibit the permeability transition pore in mouse liver mitochondria
Sensitivity of FRDA lymphoblasts to salts of transition metal ions
Frequencies of hprt(-) mutations and bcl-2 translocations in circulating human lymphocytes are correlated with United Kingdom sunlight records
The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of ...
Mitochondria in organismal aging and degeneration
  (FULL LIST AVAILABLE ON PUBMED UNDER CORTOPASSI G)