| scholarly article | Q13442814 |
| P356 | DOI | 10.1093/HMG/DDP183 |
| P8608 | Fatcat ID | release_4t4h55233bg7tk256z4ekdsmmq |
| P932 | PMC publication ID | 2694693 |
| P698 | PubMed publication ID | 19376812 |
| P5875 | ResearchGate publication ID | 229057744 |
| P50 | author | Daniel Geschwind | Q18631415 |
| Miriam Cnop | Q44930881 | ||
| Fabio Acquaviva | Q57079783 | ||
| P2093 | author name string | Sergio Cocozza | |
| Massimo Pandolfo | |||
| Giovanni Coppola | |||
| Qing Wang | |||
| Daniele Marmolino | |||
| Daning Lu | |||
| Myriam Rai | |||
| P2860 | cites work | PGC-1 coactivators: inducible regulators of energy metabolism in health and disease | Q24541524 |
| Central role of suppressors of cytokine signaling proteins in hepatic steatosis, insulin resistance, and the metabolic syndrome in the mouse | Q24564225 | ||
| Coordinated reduction of genes of oxidative metabolism in humans with insulin resistance and diabetes: Potential role of PGC1 and NRF1 | Q24679555 | ||
| Serum adiponectin as a biomarker for in vivo PPARgamma activation and PPARgamma agonist-induced efficacy on insulin sensitization/lipid lowering in rats | Q24793556 | ||
| PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis | Q24798075 | ||
| HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model | Q27301415 | ||
| Peroxisome proliferator-activated receptor gamma coactivator 1 coactivators, energy homeostasis, and metabolism | Q28266830 | ||
| PGC-1alpha: a key regulator of energy metabolism | Q28274239 | ||
| Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion | Q28275699 | ||
| Clinical and genetic abnormalities in patients with Friedreich's ataxia | Q28290611 | ||
| The cardiac phenotype induced by PPARalpha overexpression mimics that caused by diabetes mellitus | Q28344691 | ||
| Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits | Q28505173 | ||
| Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice | Q28512621 | ||
| Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia | Q28593887 | ||
| PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes | Q29547229 | ||
| Transcriptional co-activator PGC-1 alpha drives the formation of slow-twitch muscle fibres | Q29555845 | ||
| Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice | Q29614547 | ||
| Impaired mitochondrial activity in the insulin-resistant offspring of patients with type 2 diabetes | Q29614548 | ||
| Metabolic control through the PGC-1 family of transcription coactivators | Q29616509 | ||
| Dysfunction of mitochondria in human skeletal muscle in type 2 diabetes | Q29616567 | ||
| Mitochondrial dysfunction and type 2 diabetes | Q29617913 | ||
| Insulin-resistant heart exhibits a mitochondrial biogenic response driven by the peroxisome proliferator-activated receptor-alpha/PGC-1alpha gene regulatory pathway. | Q30359509 | ||
| Gene expression profiling in frataxin deficient mice: microarray evidence for significant expression changes without detectable neurodegeneration | Q33914816 | ||
| Genetic data and natural history of Friedreich's disease: a study of 80 Italian patients | Q34330412 | ||
| Disordered lipid metabolism and the pathogenesis of insulin resistance | Q34370965 | ||
| Regulation of sterol synthesis in eukaryotes. | Q34657925 | ||
| Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia | Q35653693 | ||
| Neurodegenerative disorders associated with diabetes mellitus | Q35791191 | ||
| The role of skeletal muscle insulin resistance in the pathogenesis of the metabolic syndrome | Q35901803 | ||
| Etiology of insulin resistance | Q36431215 | ||
| The Ca2+ ATPase of cardiac sarcoplasmic reticulum: Physiological role and relevance to diseases | Q36570908 | ||
| PGC-1alpha is coupled to HIF-1alpha-dependent gene expression by increasing mitochondrial oxygen consumption in skeletal muscle cells | Q37076957 | ||
| Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis | Q37526796 | ||
| Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell mass | Q39814673 | ||
| PPAR-gamma agonist Azelaoyl PAF increases frataxin protein and mRNA expression: new implications for the Friedreich's ataxia therapy. | Q39902235 | ||
| Metalloproteinase expression in PMA-stimulated THP-1 cells. Effects of peroxisome proliferator-activated receptor-gamma (PPAR gamma) agonists and 9-cis-retinoic acid. | Q40627969 | ||
| Oxidized alkyl phospholipids are specific, high affinity peroxisome proliferator-activated receptor gamma ligands and agonists | Q40816289 | ||
| Glucose metabolism alterations in Friedreich's ataxia | Q42141103 | ||
| Effects of pioglitazone on suppressor of cytokine signaling 3 expression: potential mechanisms for its effects on insulin sensitivity and adiponectin expression. | Q42830527 | ||
| Defective intracellular Ca(2+) signaling contributes to cardiomyopathy in Type 1 diabetic rats | Q44138397 | ||
| Cardiac-specific induction of the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator-1alpha promotes mitochondrial biogenesis and reversible cardiomyopathy in a developmental stage-dependent manner | Q44730585 | ||
| Measuring Friedreich ataxia: complementary features of examination and performance measures | Q44822898 | ||
| Suppressor of cytokine signaling 3 expression and insulin resistance in skeletal muscle of obese and type 2 diabetic patients | Q45032343 | ||
| Friedreich ataxia: the oxidative stress paradox | Q45198943 | ||
| Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones | Q50336075 | ||
| Quantification of the relationship between insulin sensitivity and beta-cell function in human subjects. Evidence for a hyperbolic function. | Q51598262 | ||
| Glucose intolerance in first-degree relatives of patients with Friedreich's ataxia is associated with insulin resistance: Evidence for a closely linked inherited trait | Q51609951 | ||
| Glucose and insulin metabolism in Friedreich's ataxia. | Q51669450 | ||
| Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes. | Q52541455 | ||
| Expression of SERCA isoform with faster Ca2+ transport properties improves postischemic cardiac function and Ca2+ handling and decreases myocardial infarction. | Q53540868 | ||
| Progressive loss of beta-cell function leads to worsening glucose tolerance in first-degree relatives of subjects with type 2 diabetes. | Q53568627 | ||
| Glucose intolerance in Friedreich's ataxia: association with insulin resistance and decreased insulin binding. | Q54420237 | ||
| Frataxin knockin mouse | Q57950173 | ||
| Accuracy of clinical diagnostic criteria for Friedreich's ataxia | Q62959393 | ||
| Friedreich's ataxia and oral glucose tolerance: I. The effect of ingested glucose on serum glucose and insulin values in homozygotes, obligate heterozygotes and potential carriers of the Friedreich's ataxia gene | Q67287412 | ||
| Preclinical and manifest diabetes mellitus in young patients with Friedreich's ataxia: no evidence of immune process behind the islet cell destruction | Q69522388 | ||
| Skeletal muscle NAD+(P) and NADP+-dependent malic enzyme in Friedreich's ataxia | Q71230194 | ||
| Evidence of an increased number of type IIb muscle fibers in insulin-resistant first-degree relatives of patients with NIDDM | Q73842147 | ||
| Friedreich ataxia | Q79341031 | ||
| P433 | issue | 13 | |
| P921 | main subject | Friedreich ataxia | Q913856 |
| functional genomics | Q1068690 | ||
| P304 | page(s) | 2452-2461 | |
| P577 | publication date | 2009-04-17 | |
| P1433 | published in | Human Molecular Genetics | Q2720965 |
| P1476 | title | Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARgamma pathway as a therapeutic target in Friedreich's ataxia | |
| P478 | volume | 18 |
| Q36822492 | A gene expression phenotype in lymphocytes from Friedreich ataxia patients |
| Q47071728 | Altered lipid metabolism in a Drosophila model of Friedreich's ataxia. |
| Q57905710 | An open-label trial in Friedreich ataxia suggests clinical benefit with high-dose resveratrol, without effect on frataxin levels |
| Q38121944 | Animal and cellular models of Friedreich ataxia. |
| Q36987314 | Central role and mechanisms of β-cell dysfunction and death in friedreich ataxia-associated diabetes |
| Q30613057 | Cerebellar transcriptional alterations with Purkinje cell dysfunction and loss in mice lacking PGC-1α. |
| Q36964084 | Childhood ataxia: clinical features, pathogenesis, key unanswered questions, and future directions |
| Q34452078 | Circadian rhythms, Wnt/beta-catenin pathway and PPAR alpha/gamma profiles in diseases with primary or secondary cardiac dysfunction |
| Q30581588 | Common data elements for clinical research in Friedreich's ataxia. |
| Q51555749 | Correlation of frataxin content in blood and skeletal muscle endorses frataxin as a biomarker in Friedreich ataxia. |
| Q37790615 | Current and emerging treatment options in the management of Friedreich ataxia |
| Q45863203 | Deletion of the GAA repeats from the human frataxin gene using the CRISPR-Cas9 system in YG8R-derived cells and mouse models of Friedreich ataxia |
| Q38121947 | Diabetes in Friedreich ataxia |
| Q33936940 | Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblasts |
| Q47120765 | Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia. |
| Q33877188 | Emerging functional similarities and divergences between Drosophila Spargel/dPGC-1 and mammalian PGC-1 protein |
| Q56700247 | Emerging therapeutics for the treatment of Friedreich’s ataxia |
| Q92251738 | Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia |
| Q39660098 | First Presentation of Diabetes as Diabetic Ketoacidosis in a Case of Friedreich's Ataxia |
| Q92889829 | Frataxin deficiency induces lipid accumulation and affects thermogenesis in brown adipose tissue |
| Q36541898 | Frataxin deficiency unveils cell-context dependent actions of insulin-like growth factor I on neurons |
| Q39006290 | Frataxin silencing inactivates mitochondrial Complex I in NSC34 motoneuronal cells and alters glutathione homeostasis |
| Q34076650 | Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities |
| Q36908185 | Friedreich ataxia: new pathways |
| Q34045081 | Friedreich's ataxia: the vicious circle hypothesis revisited |
| Q52931377 | Gene Expression Profile in Peripheral Blood Cells of Friedreich Ataxia Patients. |
| Q38121941 | Gene regulation and epigenetics in Friedreich's ataxia |
| Q28540063 | Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models |
| Q64044527 | Impact of Models in the Study and Treatment of Friedreich's Ataxia |
| Q30469947 | Impaired myocardial perfusion reserve and fibrosis in Friedreich ataxia: a mitochondrial cardiomyopathy with metabolic syndrome |
| Q35537136 | Increased CYFIP1 dosage alters cellular and dendritic morphology and dysregulates mTOR |
| Q47134420 | Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia |
| Q36457613 | Inflammatory mediators alter the astrocyte transcriptome and calcium signaling elicited by multiple G-protein-coupled receptors |
| Q36488119 | Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia |
| Q38034809 | Management and therapy for cardiomyopathy in Friedreich's ataxia |
| Q47072694 | Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia. |
| Q36650550 | Mitochondrial genetic diseases. |
| Q27015737 | Mitochondrial iron-sulfur cluster dysfunction in neurodegenerative disease |
| Q38121943 | Mitochondrial pathophysiology in Friedreich's ataxia |
| Q34352285 | Normal and Friedreich Ataxia Cells Express Different Isoforms of Frataxin with Complementary Roles in Iron-Sulfur Cluster Assembly |
| Q33552305 | PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia |
| Q33836935 | Past, Present and Future Therapeutics for Cerebellar Ataxias |
| Q28486160 | Pharmacological screening using an FXN-EGFP cellular genomic reporter assay for the therapy of Friedreich ataxia |
| Q39445743 | Pharmacological therapeutics in Friedreich ataxia: the present state |
| Q57138519 | Phenothiazine antioxidants increase mitochondrial biogenesis and frataxin levels in Friedreich's ataxia cells |
| Q35997389 | Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy |
| Q63090503 | Role of frataxin protein deficiency and metabolic dysfunction in Friedreich ataxia, an autosomal recessive mitochondrial disease |
| Q47168948 | Somatic instability of the expanded GAA repeats in Friedreich's ataxia. |
| Q39158402 | Therapeutic approaches for the treatment of Friedreich's ataxia |
| Q26861733 | Therapeutic developments in Friedreich ataxia |
| Q52343842 | Thermodynamics in Neurodegenerative Diseases: Interplay Between Canonical WNT/Beta-Catenin Pathway-PPAR Gamma, Energy Metabolism and Circadian Rhythms. |
| Q39239818 | Thermodynamics in cancers: opposing interactions between PPAR gamma and the canonical WNT/beta-catenin pathway |
| Q90522435 | Transcriptional profiling of isogenic Friedreich ataxia neurons and effect of an HDAC inhibitor on disease signatures |
| Q91199127 | Transcriptomic networks implicate neuronal energetic abnormalities in three mouse models harboring autism and schizophrenia-associated mutations |
| Q26860523 | Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models |
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