Mortality in Friedreich Ataxia

Although cardiac dysfunction is widely accepted as the most common cause of mortality in Friedreich ataxia (FRDA), no studies have evaluated this since the advent of specific clinical and genetic diagnostic criteria.

2021 (7)
- January (7)
  - • Dimethyl fumarate dose-depend…
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  - • Developing an Instrumented Me…
2020 (134)
- December (10)
  - • Cardiomyopathy as the first m…
  - • Effects of Fe 2+/Fe 3+ Bindin…
  - • Efficacy and Tolerability of …
  - • Exicure Provides Neuroscience…
  - • Thioredoxin and Glutaredoxin …
  - • Minoryx's clinical candidate …
  - • Calcitriol increases frataxin…
  - • Larimar Therapeutics Announce…
  - • Mitochondrial dysfunction in …
  - • Molecular Defects in Friedrei…
- November (13)
  - • Central Nervous System Therap…
  - • High Levels of Frataxin Overe…
  - • The Nrf2 induction prevents f…
  - • Antiferroptotic Activity of P…
  - • Ferroptosis in Friedreich's A…
  - • Analysis of Postural Control …
  - • PPAR gamma agonist leriglitaz…
  - • Sensitivity of Neuroimaging I…
  - • Effect of Mitochondrial and C…
  - • Electrocardiogram in Friedrei…
  - • Epicardial ablation for ventr…
  - • An Overview of the Ferroptosi…
  - • Oxidative stress modulates re…
- October (14)
  - • Aerobic Exercises Induce Anti…
  - • Safety and Efficacy of Omavel…
  - • Clinical Therapeutic Manageme…
  - • Longitudinal Study of Cogniti…
  - • Sexual function, intimate rel…
  - • Assessment of Disease Progres…
  - • Clinical Features and Molecul…
  - • Inhibition of the SUV4-20 H1 …
  - • Assessment of Ataxia Rating S…
  - • Quantitative Assessment of Fr…
  - • Oxidative stress-dependent fr…
  - • Mechanistic concepts of iron-…
  - • Expanding the genotype-phenot…
  - • Loss of mitochondrial localiz…
- September (12)
  - • Extra-mitochondrial mouse fra…
  - • A Highly Conserved Iron-Sulfu…
  - • Global, multi-stakeholder, co…
  - • Emerging therapies in Friedre…
  - • Iron-sulfur cluster protein N…
  - • Altered Secretome and ROS Pro…
  - • Iron-Sulfur Cluster Complex A…
  - • Molecular and Cellular Substr…
  - • HDAC3 deacetylates the DNA mi…
  - • Plasma and red blood cell mem…
  - • The Friedreich’s Ataxia Acc…
  - • Ataxia: Hope starts with meas…
- August (14)
  - • Friedreich Ataxia: current st…
  - • Frataxin gene editing rescues…
  - • Inherited Cerebellar Ataxias:…
  - • Atypical structures of GAA/TT…
  - • Reata provides update on deve…
  - • Test-retest reliability of th…
  - • Distribution of Particles in …
  - • Identification of a Novel Ole…
  - • R-loops promote trinucleotide…
  - • Onset features and time to di…
  - • A Drosophila model of Friedre…
  - • Antioxidant Defense Mechanism…
  - • Toward quantitative neuroimag…
  - • Antioxidant Therapies and Oxi…
- July (11)
  - • Larimar Therapeutics Announce…
  - • Relationship between activity…
  - • Current Status of microRNA-Ba…
  - • Safety and Efficacy of (+)-Ep…
  - • MR imaging and spectroscopy i…
  - • Cardiolipin-deficient cells h…
  - • Compound heterozygosity for a…
  - • Trinucleotide Repeat Disorder…
  - • Stress-induced Mouse Model of…
  - • A Novel Solution-Gated Graphe…
  - • New Request For Proposals: Th…
- June (10)
  - • Mitochondrial Damage and Sene…
  - • HMTase Inhibitors as a Potent…
  - • Feasibility and Acceptability…
  - • Treatment of Dilated Cardiomy…
  - • Defining Transcription Regula…
  - • Vestibular Impact of Friedrei…
  - • Cerebellar Cognitive Disorder…
  - • Significance of NT-proBNP and…
  - • CRISPR-Cas9 Gene Editing of H…
  - • Hereditary Ataxia: A Focus on…
- May (9)
  - • Chondrial Therapeutics and Za…
  - • Analysis of Putative Epigenet…
  - • Making iron-sulfur cluster: s…
  - • Neurofilament Light Chain as …
  - • Primary Proprioceptive Neuron…
  - • Oxidative Stress in DNA Repea…
  - • Neurologic Outcomes in Friedr…
  - • Therapeutic Potential of Stem…
  - • ExpansionHunter Denovo: A Com…
- April (10)
  - • Oxidative Stress, a Crossroad…
  - • Outlining the Complex Pathway…
  - • Progress Towards Drug Discove…
  - • A Comprehensive Transcriptome…
  - • An Overview of the Current St…
  - • Long-term voluntary running p…
  - • Calpain-Inhibitors Protect Fr…
  - • Cofilin dysregulation alters …
  - • Particle-mediated delivery of…
  - • Frataxin-deficient cardiomyoc…
- March (7)
  - • A wearable video-oculography …
  - • Neurophysiologic intraoperati…
  - • Design Therapeutics Launches …
  - • Identification of Frataxin as…
  - • The Assessment of Upper Limb …
  - • Inherited Metabolic Diseases …
  - • Management of Neuroinflammato…
- February (8)
  - • Psychometric properties of th…
  - • On the wrong DNA track: Molec…
  - • The Potential of the Novel NA…
  - • Ocular Involvement in Friedre…
  - • The NRF2 Signaling Network De…
  - • Mechanisms of Mitochondrial I…
  - • Developing an objective evalu…
  - • NfL and pNfH are increased in…
- January (16)
  - • Correlation of Visual Quality…
  - • Frataxin deficiency induces l…
  - • Imaging neuronal activity in …
  - • An Instrumented Measurement S…
  - • Predictors of loss of ambulat…
  - • Frataxin Structure and Functi…
  - • Safety and Efficacy of Interf…
  - • Frataxin deficiency in Friedr…
  - • Health related quality of lif…
  - • Multiple mechanisms underpin …
  - • Large-scale contractions of F…
  - • MDA Awards Venture Philanthro…
  - • Mammalian iron-sulfur cluster…
  - • Retrotope Expands its Drug Pi…
  - • Longitudinal Increases in Cer…
  - • Genetic Analysis of Hereditar…
2019 (102)
- December (16)
  - • Exenatide induces frataxin ex…
  - • Nanoscopic X-ray imaging and …
  - • Age of onset determines intri…
  - • A new tool to determine the c…
  - • Electrophysiological evidence…
  - • Neurochemical profiles in her…
  - • Iron Hack - A symposium/hacka…
  - • Chondrial Therapeutics Announ…
  - • Zafgen and Chondrial Therapeu…
  - • Exicure Announces First Neuro…
  - • Effects of tocotrienol supple…
  - • Friedreich's Ataxia: Case ser…
  - • Mitochondrial dysfunction in …
  - • Pediatric Neuromuscular Disor…
  - • N-Terminomics/TAILS Profiling…
  - • Possible Mechanisms of Biolog…
- November (11)
  - • Left ventricular structural a…
  - • Global Ataxia Advocacy Organi…
  - • Orphan Drugs In Development F…
  - • Temporal but not spatial dysm…
  - • Quantitative Proteomic and Ne…
  - • Refinement of protein Fe(II) …
  - • Evolutionarily conserved susc…
  - • Speech and Language Disorders…
  - • The Structure of the Human AC…
  - • Potential biomarker identific…
  - • The first biallelic missense …
- October (11)
  - • Targeting NRF2 for the Treatm…
  - • Cerebellum and cognition in F…
  - • Predictors of Left Ventricula…
  - • Induced pluripotent stem cell…
  - • Minoryx Therapeutics receives…
  - • Reata Announces Positive Topl…
  - • Primary Cultures of Pure Embr…
  - • Changes detected in swallowin…
  - • SINEUP non-coding RNAs rescue…
  - • Minoryx Therapeutics complete…
  - • Health-related quality of lif…
- September (6)
  - • Unique roles of iron and zinc…
  - • Mechanism of activation of th…
  - • Two new Pfizer-coauthored stu…
  - • Scoliosis in Patients With Fr…
  - • Friedreich ataxia- pathogenes…
  - • The Working Life of People wi…
- August (7)
  - • Backbone resonance assignment…
  - • Application of Quantitative M…
  - • Oral mobility reflects rate o…
  - • Excision of the expanded GAA …
  - • Folding and Dynamics are Stro…
  - • Nrf2 Induction Re-establishes…
  - • Physiologically relevant reco…
- July (8)
  - • Exploring iron-binding to hum…
  - • Low frataxin mRNA expression …
  - • REGENXBIO Announces New Licen…
  - • Molecular Mechanisms and Ther…
  - • New developments in pharmacot…
  - • The current state of biomarke…
  - • Correlation between frataxin …
  - • Evaluation of antibodies for …
- June (11)
  - • Frequency and Genetic Profile…
  - • FARA Job Posting: Associate D…
  - • Evidence for genetically dete…
  - • Open label Pilot Study of Ora…
  - • Deuterated Polyunsaturated Fa…
  - • Altered neocortical tactile b…
  - • Young Investigators: Call for…
  - • Microvascular pathology in Fr…
  - • p53 Binds Preferentially to N…
  - • Dimethyl fumarate dosing in h…
  - • Efficient Electroporation of …
- May (5)
- April (5)
- March (7)
  - • StrideBio and Takeda Sign Col…
  - • Patient reported outcomes in …
  - • Assessment of cell-free level…
  - • Inherited Ataxia and Intrathe…
  - • The role of robotic gait trai…
  - • The Role of Iron in Friedreic…
  - • Progress in understanding Fri…
- February (8)
  - • C-Path and FARA announce the …
  - • Minoryx Therapeutics receives…
  - • Guidelines on the diagnosis a…
  - • In Vitro interaction between …
  - • The role of mitochondrial lab…
  - • Pattern of Cerebellar Atrophy…
  - • Phosphodiesterase Inhibitors …
  - • Heart disease in Friedreich's…
- January (7)
  - • Neurocrine Biosciences and Vo…
  - • OGTT is recommended for gluco…
  - • Ferroptosis as a novel therap…
  - • Structural and functional cha…
  - • Longitudinal evaluation of ir…
  - • Drug repositioning screening …
  - • Differences in the determinan…
2018 (68)
- December (5)
- November (7)
  - • FAST-1 antisense RNA epigenet…
  - • Characterization of a new N-t…
  - • Rapid and Complete Reversal o…
  - • Oligonucleotides Hold Promise…
  - • Voyager Therapeutics Announce…
  - • Safety, pharmacodynamics, and…
  - • Plasma Markers of Neurodegene…
- October (6)
  - • Intraepidermal Nerve Fiber De…
  - • Calcium Deregulation: Novel I…
  - • Developmental and Neurodegene…
  - • Corneal Confocal Microscopy: …
  - • Phenothiazine antioxidants in…
  - • Automated functional upper li…
- September (5)
- August (6)
  - • Test-retest reliability of an…
  - • Two funded post-doctoral posi…
  - • Nonataxia symptoms in Friedre…
  - • Activating frataxin expressio…
  - • Novel Nrf2-Inducer Prevents M…
  - • Longitudinal analysis of cont…
- July (7)
  - • RNA-Dependent Epigenetic Sile…
  - • Zinc(II) binding on human wil…
  - • PTC Therapeutics to acquire A…
  - • Young Investigators, Call for…
  - • Impact of Drosophila Models i…
  - • Autonomic function testing in…
  - • Structural characteristics of…
- June (5)
- May (5)
- April (3)
- March (9)
  - • Identification of p38 MAPK as…
  - • Iron regulatory protein defic…
  - • Mitofusin-Dependent ER Stress…
  - • GAA•TTC repeat expansion in…
  - • Pitfalls in molecular diagnos…
  - • Bone marrow transplantation s…
  - • Biophysical characterisation …
  - • Small RNA-seq analysis of cir…
  - • Progress in the treatment of …
- February (3)
- January (7)
  - • Activation of Frataxin Protei…
  - • Join Reata WebEx. Get updates…
  - • Quantitative proteomics in Fr…
  - • The role of Nrf2 signaling in…
  - • Effect Of Diazoxide on Friedr…
  - • Emotion Recognition and Psych…
  - • Friedreich and dominant ataxi…
2017 (79)
- December (8)
  - • Liquid Chromatography-High Re…
  - • Early VGLUT1-specific paralle…
  - • Somatic instability of the ex…
  - • Inducible and reversible phen…
  - • Mitochondrial pore opening an…
  - • The role of oxidative stress …
  - • Synthetic transcription elong…
  - • C-Path and FARA announce coll…
- November (5)
- October (16)
  - • Agilis Biotherapeutics Announ…
  - • Can rehabilitation improve th…
  - • Transplantation of wild-type …
  - • Friedreich's ataxia: clinical…
  - • Nrf2-Inducers Counteract Neur…
  - • Reata Announces First Patient…
  - • Friedreich Ataxia: Developmen…
  - • Sustained FXN expression in d…
  - • BioMarin Highlights Breadth o…
  - • CRISPR Therapeutics Awarded G…
  - • IARC 2017 Follow Up
  - • The Subclinical Cardiomyopath…
  - • FDA awards six grants for nat…
  - • Frataxin-deficient neurons an…
  - • Urinary, bowel and sexual sym…
  - • Agilis Biotherapeutics Update…
- September (6)
  - • Molecular Alterations in a Mo…
  - • Lipophilic methylene violet a…
  - • Reversible Axonal Dystrophy b…
  - • Impact of diabetes in the Fri…
  - • LNA effects on DNA binding an…
  - • Defining the Architecture of …
- August (10)
  - • Peptide SS-31 upregulates fra…
  - • Overexpression of Drosophila …
  - • Part 2 of the Phase II MOXIe …
  - • Cerebral Abnormalities in Fri…
  - • Jupiter Orphan Therapeutics R…
  - • Selected missense mutations i…
  - • FDA Confirms That Use of mFAR…
  - • Heart and Nervous System Path…
  - • Mitochondrial dysfunction in …
  - • Chondrial Announces FDA Orpha…
- July (6)
  - • Pharmacological therapeutics …
  - • Nicotinamide mononucleotide r…
  - • Call for Abstracts - 10th Ann…
  - • AFAF and FARA invite proposal…
  - • Circulating miR-323-3p is a b…
  - • How does performance of the F…
- June (2)
  - • Friedreich's ataxia induced p…
  - • Reata Pharmaceuticals, Inc. A…
- May (8)
  - • Cerebral compensation during …
  - • Progressive mitochondrial pro…
  - • Structural signature of class…
  - • Erythropoietin and small mole…
  - • Dimethyl Fumarate Mediates Nr…
  - • Normalization of timed neurop…
  - • Dysphagia in Friedreich Ataxi…
  - • Mesenchymal Stem Cell-Derived…
- April (3)
- March (4)
- February (4)
- January (7)
  - • Cardiac transplantation in Fr…
  - • Newly Funded FARA Grants
  - • Friedreich Ataxia: Hypoplasia…
  - • STEADFAST Background for the …
  - • Cytokine therapy-mediated neu…
  - • Deletion of the GAA repeats f…
  - • Mitochondrial capacity, muscl…
2016 (87)
- December (3)
- November (4)
- September (9)
  - • A longitudinal study of the S…
  - • Progression of Friedreich ata…
  - • Retrotope announces Phase I/I…
  - • Alleviating GAA repeat induce…
  - • Cognition in Friedreich's ata…
  - • Friedreich ataxia induced plu…
  - • Functional and Gait Assessmen…
  - • Pharmacological treatments fo…
  - • Neurobehavioral deficits in t…
- August (9)
  - • Serum versus Imaging Biomarke…
  - • Lentivirus-meditated frataxin…
  - • Frataxin silencing alters mic…
  - • Architecture of the Human Mit…
  - • Cerebral and cerebellar grey …
  - • Voice in Friedreich Ataxia
  - • Long-term treatment with thia…
  - • Agilis Biotherapeutics Announ…
  - • Pfizer aims to become industr…
- July (9)
  - • 9th Annual Friedreich’s Ata…
  - • The Replication of Frataxin G…
  - • Metal Homeostasis Regulators …
  - • The significance of intercala…
  - • Interferon gamma may improve …
  - • Deep sequencing of mitochondr…
  - • Crystal Structure of Bacillus…
  - • Downregulation of GSTK1 Is a …
  - • Vestibulo-ocular reflex dynam…
- June (3)
- May (11)
  - • Mitochondrial energy imbalanc…
  - • Reata Pharmaceuticals, Inc. A…
  - • Purkinje cell injury, structu…
  - • Plasma circulating cell-free …
  - • Horizon Pharma plc to Acquire…
  - • Abundance and Significance of…
  - • Meeting Notice: FARA's Bioma…
  - • Job Opportunity: Postdoctoral…
  - • Dorsal root ganglia in Friedr…
  - • Horizon Pharma plc Completes …
  - • Frataxin and the molecular me…
- April (11)
  - • Time-resolved functional anal…
  - • Two different pathogenic mech…
  - • Development of an AAV9 coding…
  - • Comorbid Medical Conditions i…
  - • Lymphoblast Oxidative Stress …
  - • Effects of Genetic Severity o…
  - • Using human pluripotent stem …
  - • Gastrocnemius and soleus spas…
  - • The Structure of the Complex …
  - • Clinical Experience With Defe…
  - • Iron chelation in the treatme…
- March (4)
- February (8)
  - • Reversal of epigenetic promot…
  - • Combined Cerebellar Proton MR…
  - • Intrathecal delivery of frata…
  - • Long-term effect of epoetin a…
  - • Retrotope advances RT001 in c…
  - • Gene transfer of brain derive…
  - • Activating frataxin expressio…
  - • Avalanche Biotechnologies and…
- January (16)
  - • Emerging therapies in Friedre…
  - • Friedreich Ataxia and nephrot…
  - • Nonneurological Involvement i…
  - • Pathology of Intercalated Dis…
  - • FARA Announces Catabasis Phar…
  - • Update on the STEADFAST study
  - • Chondrial Therapeutics Announ…
  - • Jupiter Orphan Therapeutics, …
  - • FARA Grant Program Update
  - • Annapurna Therapeutics to Col…
  - • Compound heterozygous FXN mut…
  - • Longitudinal magnetic resonan…
  - • Perturbation of cellular prot…
  - • Analyzing the Effects of a G1…
  - • GIFT-1, a phase IIa clinical …
  - • Efficient attenuation of Frie…
2015 (23)
- December (2)
  - • Retrotope RT0001 Clinical Tri…
  - • Reata RTA 408 Clinical Trial …
- November (2)
  - • Retrotope announces opening o…
  - • Funding Announcement: Clinica…
- September (8)
  - • A 22-Year Follow-up Study of …
  - • Expanded GAA repeats impede t…
  - • FXN Promoter Silencing in the…
  - • Delayed-onset Friedreich's at…
  - • Evidence for chromosome fragi…
  - • Frataxin levels in peripheral…
  - • Friedreich ataxia in Norway -…
  - • Investigation of mitochondria…
- August (11)
  - • Stable isotopes and LC-MS for…
  - • Phenotypic Screening for Frie…
  - • Key Patent Granted For AAVLif…
  - • New funding opportunity annou…
  - • Call for Abstracts due Friday…
  - • Targeting lipid peroxidation …
  - • Mitigation of Myocardial Isch…
  - • The Role of Frataxin in Doxor…
  - • Regional Cerebral Disease Pro…
  - • Gene Expression Profile in Pe…
  - • TORC1 Inhibition by Rapamycin…

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Mortality in Friedreich Ataxia

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