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Search results 1 to 100 out of 264 for Fxn

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0.038s
Type Details Score
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: chicken
Gene
Type: gene
Organism: zebrafish
Gene
Type: gene
Organism: macaque, rhesus
Gene
Type: gene
Organism: frog, western clawed
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: dog, domestic
Gene
Type: gene
Organism: chimpanzee
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Publication
First Author: Chutake YK
Year: 2015
Journal: PLoS One
Title: FXN Promoter Silencing in the Humanized Mouse Model of Friedreich Ataxia.
Volume: 10
Issue: 9
Pages: e0138437
Publication  
First Author: Filali M
Year: 2015
Journal: Brain Res
Title: Sensorimotor skills in Fxn KO/Mck mutants deficient for frataxin in muscle.
Volume: 1608
Pages: 91-6
Publication
First Author: Medina-Carbonero M
Year: 2022
Journal: Cell Mol Life Sci
Title: Mice harboring the FXN I151F pathological point mutation present decreased frataxin levels, a Friedreich ataxia-like phenotype, and mitochondrial alterations.
Volume: 79
Issue: 2
Pages: 74
Publication  
First Author: Miller JL
Year: 2017
Journal: Neuropharmacology
Title: Erythropoietin and small molecule agonists of the tissue-protective erythropoietin receptor increase FXN expression in neuronal cells in vitro and in Fxn-deficient KIKO mice in vivo.
Volume: 123
Pages: 34-45
Publication
First Author: Tomassini B
Year: 2012
Journal: Hum Mol Genet
Title: Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model.
Volume: 21
Issue: 13
Pages: 2855-61
Publication
First Author: Rodden LN
Year: 2022
Journal: Sci Rep
Title: DNA methylation in Friedreich ataxia silences expression of frataxin isoform E.
Volume: 12
Issue: 1
Pages: 5031
Publication  
First Author: Villa C
Year: 2021
Journal: Sci Transl Med
Title: Treatment with ROS detoxifying gold quantum clusters alleviates the functional decline in a mouse model of Friedreich ataxia.
Volume: 13
Issue: 607
Publication
First Author: Sandi C
Year: 2011
Journal: Neurobiol Dis
Title: Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model.
Volume: 42
Issue: 3
Pages: 496-505
Publication
First Author: Mollá B
Year: 2016
Journal: Dis Model Mech
Title: Two different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich's ataxia.
Volume: 9
Issue: 6
Pages: 647-57
Publication  
First Author: Abeti R
Year: 2022
Journal: Front Mol Biosci
Title: A Drug Combination Rescues Frataxin-Dependent Neural and Cardiac Pathophysiology in FA Models.
Volume: 9
Pages: 830650
Publication
First Author: Sahdeo S
Year: 2014
Journal: Hum Mol Genet
Title: Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.
Volume: 23
Issue: 25
Pages: 6848-62
Publication  
First Author: Kalef-Ezra E
Year: 2023
Journal: Front Neurosci
Title: A new FRDA mouse model [Fxn (null):YG8s(GAA) > 800] with more than 800 GAA repeats.
Volume: 17
Pages: 930422
Publication
First Author: Liu Y
Year: 2021
Journal: Hum Mol Genet
Title: SS-31 efficacy in a mouse model of Friedreich ataxia by upregulation of frataxin expression.
Volume: 31
Issue: 2
Pages: 176-188
Publication  
First Author: Gérard C
Year: 2023
Journal: Behav Brain Res
Title: A promising mouse model for Friedreich Ataxia progressing like human patients.
Volume: 436
Pages: 114107
Publication
First Author: Al-Mahdawi S
Year: 2008
Journal: Hum Mol Genet
Title: The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues.
Volume: 17
Issue: 5
Pages: 735-46
Publication  
First Author: Rocca CJ
Year: 2017
Journal: Sci Transl Med
Title: Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia.
Volume: 9
Issue: 413
Publication
First Author: Anjomani Virmouni S
Year: 2014
Journal: PLoS One
Title: Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia.
Volume: 9
Issue: 9
Pages: e107416
Publication
First Author: Al-Mahdawi S
Year: 2006
Journal: Genomics
Title: GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology.
Volume: 88
Issue: 5
Pages: 580-90
Publication
First Author: Anjomani Virmouni S
Year: 2015
Journal: Dis Model Mech
Title: A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxia.
Volume: 8
Issue: 3
Pages: 225-35
Publication  
First Author: Franco C
Year: 2012
Journal: Mol Neurodegener
Title: Frataxin deficiency unveils cell-context dependent actions of insulin-like growth factor I on neurons.
Volume: 7
Pages: 51
Publication
First Author: Turchi R
Year: 2023
Journal: iScience
Title: Butyrate prevents visceral adipose tissue inflammation and metabolic alterations in a Friedreich's ataxia mouse model.
Volume: 26
Issue: 10
Pages: 107713
Publication
First Author: Santoro A
Year: 2018
Journal: Hum Mol Genet
Title: Effect of diazoxide on Friedreich ataxia models.
Volume: 27
Issue: 6
Pages: 992-1001
Publication
First Author: Shan Y
Year: 2013
Journal: Antioxid Redox Signal
Title: Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model.
Volume: 19
Issue: 13
Pages: 1481-93
Publication
First Author: Pook MA
Year: 2001
Journal: Neurogenetics
Title: Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis.
Volume: 3
Issue: 4
Pages: 185-93
Publication  
First Author: Rodríguez-Pascau L
Year: 2021
Journal: Neurobiol Dis
Title: PPAR gamma agonist leriglitazone improves frataxin-loss impairments in cellular and animal models of Friedreich Ataxia.
Volume: 148
Pages: 105162
Publication  
First Author: Igoillo-Esteve M
Year: 2020
Journal: JCI Insight
Title: Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia.
Volume: 5
Issue: 2
Publication
First Author: Cotticelli MG
Year: 2019
Journal: J Pharmacol Exp Ther
Title: Ferroptosis as a Novel Therapeutic Target for Friedreich's Ataxia.
Volume: 369
Issue: 1
Pages: 47-54
Publication
First Author: Zhao H
Year: 2020
Journal: Sci Rep
Title: Long-term voluntary running prevents the onset of symptomatic Friedreich's ataxia in mice.
Volume: 10
Issue: 1
Pages: 6095
Publication
First Author: Martelli A
Year: 2012
Journal: Dis Model Mech
Title: Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia.
Volume: 5
Issue: 6
Pages: 860-9
Publication
First Author: Vyas PM
Year: 2012
Journal: Hum Mol Genet
Title: A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model.
Volume: 21
Issue: 6
Pages: 1230-47
Publication  
First Author: Culley MK
Year: 2021
Journal: J Clin Invest
Title: Frataxin deficiency promotes endothelial senescence in pulmonary hypertension.
Volume: 131
Issue: 11
Publication
First Author: Perdomini M
Year: 2014
Journal: Nat Med
Title: Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia.
Volume: 20
Issue: 5
Pages: 542-7
Publication  
First Author: Franco C
Year: 2017
Journal: Mol Cell Neurosci
Title: A role for astrocytes in cerebellar deficits in frataxin deficiency: Protection by insulin-like growth factor I.
Volume: 80
Pages: 100-110
Publication
First Author: Chang JC
Year: 2024
Journal: Mol Ther Methods Clin Dev
Title: AAV8 gene therapy reverses cardiac pathology and prevents early mortality in a mouse model of Friedreich's ataxia.
Volume: 32
Issue: 1
Pages: 101193
Publication
First Author: Jasoliya MJ
Year: 2017
Journal: Hum Mol Genet
Title: Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans.
Volume: 26
Issue: 14
Pages: 2627-2633
Publication
First Author: Turchi R
Year: 2020
Journal: Cell Death Dis
Title: Frataxin deficiency induces lipid accumulation and affects thermogenesis in brown adipose tissue.
Volume: 11
Issue: 1
Pages: 51
Publication
First Author: Sarsero JP
Year: 2014
Journal: PLoS One
Title: Rescue of the Friedreich ataxia knockout mutation in transgenic mice containing an FXN-EGFP genomic reporter.
Volume: 9
Issue: 3
Pages: e93307
Publication  
First Author: Fil D
Year: 2020
Journal: Dis Model Mech
Title: Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia.
Volume: 13
Issue: 7
Publication
First Author: Schultz R
Year: 2016
Journal: Invest Ophthalmol Vis Sci
Title: Increased Frataxin Levels Protect Retinal Ganglion Cells After Acute Ischemia/Reperfusion in the Mouse Retina In Vivo.
Volume: 57
Issue: 10
Pages: 4115-24
Publication
First Author: Hayashi G
Year: 2014
Journal: Hum Mol Genet
Title: Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia.
Volume: 23
Issue: 25
Pages: 6838-47
Publication  
First Author: Al-Mahdawi S
Year: 2018
Journal: Front Cell Neurosci
Title: Large Interruptions of GAA Repeat Expansion Mutations in Friedreich Ataxia Are Very Rare.
Volume: 12
Pages: 443
Publication
First Author: Al-Mahdawi S
Year: 2004
Journal: Genomics
Title: GAA repeat instability in Friedreich ataxia YAC transgenic mice.
Volume: 84
Issue: 2
Pages: 301-10
Publication  
First Author: Muñoz-Lasso DC
Year: 2022
Journal: Front Mol Neurosci
Title: Frataxin Deficit Leads to Reduced Dynamics of Growth Cones in Dorsal Root Ganglia Neurons of Friedreich's Ataxia YG8sR Model: A Multilinear Algebra Approach.
Volume: 15
Pages: 912780
Publication
First Author: Muñoz-Lasso DC
Year: 2020
Journal: Sci Rep
Title: Cofilin dysregulation alters actin turnover in frataxin-deficient neurons.
Volume: 10
Issue: 1
Pages: 5207
Publication
First Author: Stram AR
Year: 2017
Journal: PLoS One
Title: Progressive mitochondrial protein lysine acetylation and heart failure in a model of Friedreich's ataxia cardiomyopathy.
Volume: 12
Issue: 5
Pages: e0178354
Publication  
First Author: Fil D
Year: 2023
Journal: Neurobiol Dis
Title: Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin.
Volume: 177
Pages: 105996
Publication
First Author: Martelli A
Year: 2015
Journal: Cell Metab
Title: Iron regulatory protein 1 sustains mitochondrial iron loading and function in frataxin deficiency.
Volume: 21
Issue: 2
Pages: 311-22
Publication
First Author: Jones J
Year: 2013
Journal: PLoS One
Title: Stem cells from wildtype and Friedreich's ataxia mice present similar neuroprotective properties in dorsal root ganglia cells.
Volume: 8
Issue: 5
Pages: e62807
Publication
First Author: Whitnall M
Year: 2008
Journal: Proc Natl Acad Sci U S A
Title: The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation.
Volume: 105
Issue: 28
Pages: 9757-62
Protein
Organism: Mus musculus/domesticus
Length: 207  
Fragment?: false
Publication
First Author: Martelli A
Year: 2007
Journal: Hum Mol Genet
Title: Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues.
Volume: 16
Issue: 22
Pages: 2651-8
Publication
First Author: Roy AR
Year: 2018
Journal: J Biol Chem
Title: The transcriptional regulator CCCTC-binding factor limits oxidative stress in endothelial cells.
Volume: 293
Issue: 22
Pages: 8449-8461
Publication
First Author: Oktay Y
Year: 2007
Journal: J Biol Chem
Title: Hypoxia-inducible factor 2alpha regulates expression of the mitochondrial aconitase chaperone protein frataxin.
Volume: 282
Issue: 16
Pages: 11750-6
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus spretus
Publication
First Author: Koutnikova H
Year: 1997
Journal: Nat Genet
Title: Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin.
Volume: 16
Issue: 4
Pages: 345-51
Publication
First Author: Calmels N
Year: 2009
Journal: PLoS One
Title: The first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxia.
Volume: 4
Issue: 7
Pages: e6379
Publication
First Author: Huang ML
Year: 2009
Journal: Proc Natl Acad Sci U S A
Title: Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant.
Volume: 106
Issue: 38
Pages: 16381-6
Publication
First Author: Coppola G
Year: 2006
Journal: Neurobiol Dis
Title: Gene expression profiling in frataxin deficient mice: microarray evidence for significant expression changes without detectable neurodegeneration.
Volume: 22
Issue: 2
Pages: 302-11
Publication
First Author: McMackin MZ
Year: 2019
Journal: PLoS One
Title: Potential biomarker identification for Friedreich's ataxia using overlapping gene expression patterns in patient cells and mouse dorsal root ganglion.
Volume: 14
Issue: 10
Pages: e0223209
Publication
First Author: Lin H
Year: 2017
Journal: Dis Model Mech
Title: Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia.
Volume: 10
Issue: 11
Pages: 1343-1352
Publication
First Author: Puccio H
Year: 2001
Journal: Nat Genet
Title: Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits.
Volume: 27
Issue: 2
Pages: 181-6
Publication
First Author: Whitnall M
Year: 2012
Journal: Proc Natl Acad Sci U S A
Title: Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia.
Volume: 109
Issue: 50
Pages: 20590-5
Publication
First Author: Anzovino A
Year: 2017
Journal: Am J Pathol
Title: Molecular Alterations in a Mouse Cardiac Model of Friedreich Ataxia: An Impaired Nrf2 Response Mediated via Upregulation of Keap1 and Activation of the Gsk3β Axis.
Volume: 187
Issue: 12
Pages: 2858-2875
Publication
First Author: Simon D
Year: 2004
Journal: J Neurosci
Title: Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia.
Volume: 24
Issue: 8
Pages: 1987-95
Publication
First Author: Seznec H
Year: 2004
Journal: Hum Mol Genet
Title: Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia.
Volume: 13
Issue: 10
Pages: 1017-24
Publication
First Author: Belbellaa B
Year: 2019
Journal: Hum Mol Genet
Title: Correction of half the cardiomyocytes fully rescue Friedreich ataxia mitochondrial cardiomyopathy through cell-autonomous mechanisms.
Volume: 28
Issue: 8
Pages: 1274-1285
Publication
First Author: Thierbach R
Year: 2005
Journal: Hum Mol Genet
Title: Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice.
Volume: 14
Issue: 24
Pages: 3857-64
Publication
First Author: Pomplun D
Year: 2007
Journal: Proc Natl Acad Sci U S A
Title: Reduced expression of mitochondrial frataxin in mice exacerbates diet-induced obesity.
Volume: 104
Issue: 15
Pages: 6377-81
Publication
First Author: Thierbach R
Year: 2012
Journal: Hum Mol Genet
Title: Specific alterations of carbohydrate metabolism are associated with hepatocarcinogenesis in mitochondrially impaired mice.
Volume: 21
Issue: 3
Pages: 656-63
Publication
First Author: Poburski D
Year: 2016
Journal: Biol Open
Title: Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia.
Volume: 5
Issue: 5
Pages: 654-61
Publication  
First Author: Chiang S
Year: 2020
Journal: Pharmacol Res
Title: The potential of the novel NAD+ supplementing agent, SNH6, as a therapeutic strategy for the treatment of Friedreich's ataxia.
Volume: 155
Pages: 104680
Publication
First Author: Ristow M
Year: 2003
Journal: J Clin Invest
Title: Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell mass.
Volume: 112
Issue: 4
Pages: 527-34