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Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 9/2009, Volume 106, Issue 38, pp. 16381 - 16386
We used the muscle creatine kinase (MCK) conditional frataxin knockout mouse to elucidate how frataxin deficiency alters iron metabolism. This is of... 
Up regulation | Molecules | Mitochondria | Energy metabolism | Messenger RNA | Reverse transcriptase polymerase chain reaction | Lead | Iron | Friedreich ataxia | Down regulation | Frataxin | Iron transferrin | Heme synthesis | CELLS | heme synthesis | FRATAXIN HOMOLOG | HEME-SYNTHESIS | MULTIDISCIPLINARY SCIENCES | transferrin | INTERLEUKIN-6 | DEFICIENCY | frataxin | CLUSTER SYNTHESIS | METABOLISM | iron | MICE | PROTEINS | EXPRESSION | Heme - metabolism | Oligonucleotide Array Sequence Analysis | Friedreich Ataxia - pathology | Humans | Gene Expression Profiling | Antimicrobial Cationic Peptides - metabolism | Iron-Binding Proteins - metabolism | Friedreich Ataxia - genetics | Kidney - metabolism | Carbon-Sulfur Lyases - genetics | Hepcidins | Myocardium - metabolism | Antimicrobial Cationic Peptides - genetics | Disease Models, Animal | Friedreich Ataxia - metabolism | Liver - metabolism | Uroporphyrinogen III Synthetase - genetics | Uroporphyrinogen III Synthetase - metabolism | Mitochondria - metabolism | Coproporphyrinogen Oxidase - genetics | Reverse Transcriptase Polymerase Chain Reaction | Carbon-Sulfur Lyases - metabolism | Iron - metabolism | Blotting, Western | Mice, Knockout | Myocardium - cytology | Animals | Spleen - metabolism | Ferrochelatase - metabolism | Ferrochelatase - genetics | Iron-Binding Proteins - genetics | Mice | Coproporphyrinogen Oxidase - metabolism | Tissue | Rodents | Vitamin deficiency | Mutation | Kinases | Metabolism | Index Medicus | Biological Sciences
Journal Article
PLoS ONE, ISSN 1932-6203, 03/2015, Volume 10, Issue 3, pp. e0116396 - e0116396
Friedreich ataxia (FA) is an autosomal recessive disease with a complex neurological phenotype, but the most common cause of death is heart failure. This study... 
HYPERTROPHY | HEARTS | MITOCHONDRIAL FERRITIN | INFLAMMATION | MULTIDISCIPLINARY SCIENCES | DISEASE | LIVER | HEPCIDIN | IRON | ATHEROSCLEROTIC PLAQUE | LIFE | Hepcidins - metabolism | Friedreich Ataxia - metabolism | Mitochondria, Heart - metabolism | Friedreich Ataxia - pathology | Humans | Middle Aged | Male | Iron - metabolism | Case-Control Studies | Young Adult | Ferritins - metabolism | Myocardium - metabolism | Adolescent | Myocarditis - metabolism | Adult | Female | Aged | Heart Ventricles - pathology | Care and treatment | Usage | Cardiomyopathy | Patient outcomes | Ataxia | Genetic aspects | Research | Diagnosis | X-ray spectroscopy | Heart diseases | Immunohistochemistry | Heart | Health sciences | Neurosciences | Transcription | Veterans | Personal health | Pathogenesis | X ray fluorescence | Ferritin | Fluorescence | Iron | Macrophages | Tissues | Accumulation | Spectrometry | Mitochondria | Frataxin | Autopsy | Polyethylene glycol | Failure analysis | Bioindicators | Localization | Deoxyribonucleic acid--DNA | Polyethylene | Autopsies | Antiinflammatory agents | Environmental health | Septum | Cardiomyocytes | Inflammation | Patients | Zinc | Myocarditis | Pathology | Friedreich's ataxia | Inductively coupled plasma | X-ray fluorescence | Fibrosis | Weight reduction | Ventricle | Immunofluorescence | Hepcidin | Histochemistry | Hypertrophy | Index Medicus | Deoxyribonucleic acid | DNA
Journal Article
PLoS Biology, ISSN 1544-9173, 03/2012, Volume 10, Issue 3, pp. e1001288 - e1001288
An increasing number of genes required for mitochondrial biogenesis, dynamics, or function have been found to be mutated in metabolic disorders and... 
RETINAL DEGENERATION | DNA DEPLETION | DROSOPHILA-PARKIN MUTANTS | UNFOLDED PROTEIN RESPONSE | PINK1/PARKIN PATHWAY | GENE | FRIEDREICHS-ATAXIA | COPY-NUMBER | GENOMIC HYBRIDIZATION-ARRAY | BIOCHEMISTRY & MOLECULAR BIOLOGY | DISEASE | BIOLOGY | Mitochondria - enzymology | Electron Transport | Electroretinography - methods | Leukoencephalopathies - genetics | Retina - metabolism | Cell Proliferation | Reactive Oxygen Species - metabolism | Humans | Middle Aged | Muscles - physiopathology | Child, Preschool | Male | Mitochondrial Proteins - genetics | Drosophila Proteins - metabolism | Drosophila - physiology | Drosophila - enzymology | Young Adult | Mitochondria - genetics | Mitochondrial Proteins - metabolism | HEK293 Cells | Adult | Female | Methionine-tRNA Ligase - metabolism | Photoreceptor Cells - pathology | Ataxia - genetics | Muscles - metabolism | Ataxia - metabolism | Child | Drosophila - genetics | Oxidative Phosphorylation | Neurodegenerative Diseases - genetics | Leukoencephalopathies - metabolism | Longevity | Neurodegenerative Diseases - metabolism | Unfolded Protein Response | Gene Expression Regulation, Enzymologic | Phenotype | Animals | Pedigree | Photoreceptor Cells - metabolism | Adolescent | Drosophila Proteins - genetics | Mutation | Methionine-tRNA Ligase - genetics | Retina - pathology | Proteins | Medical research | Genetics | Neurodegeneration | Genes | Index Medicus
Journal Article
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 12/2012, Volume 109, Issue 50, pp. 20590 - 20595
There is no effective treatment for the cardiomyopathy of the most common autosomal recessive ataxia, Friedreich ataxia (FA). This disease is due to decreased... 
Heart | Protein metabolism | Energy metabolism | Diet | Liver | Cardiomyopathies | Mice | Down regulation | Ferritins | Skeletal muscle | Ferroportin 1 | Hemojuvelin | Transferrin receptor 1 | Heme oxygenase | heme oxygenase | MECHANISM | HEME-SYNTHESIS | MULTIDISCIPLINARY SCIENCES | CARDIOMYOPATHY | TRAFFICKING | hemojuvelin | RETICULOCYTES | ferroportin 1 | SUCCINYLACETONE | METABOLISM | transferrin receptor 1 | TISSUES | FERRITIN | PROTEINS | Mitochondria, Heart - metabolism | Creatine Kinase, MM Form - metabolism | Friedreich Ataxia - pathology | Humans | Cardiomegaly - pathology | Iron, Dietary - administration & dosage | Iron-Binding Proteins - metabolism | Friedreich Ataxia - genetics | Mice, Mutant Strains | Myocardium - metabolism | Disease Models, Animal | Iron - blood | Myocardium - ultrastructure | Microscopy, Electron, Transmission | Friedreich Ataxia - metabolism | Creatine Kinase, MM Form - genetics | Signal Transduction | Spectroscopy, Mossbauer | Liver - metabolism | Iron - metabolism | Mice, Knockout | Animals | Cardiomegaly - prevention & control | Iron-Binding Proteins - antagonists & inhibitors | Iron-Binding Proteins - genetics | Iron Regulatory Protein 2 - metabolism | Cardiomegaly - metabolism | Physiological aspects | Ataxia | Development and progression | Iron in the body | Metabolism | Health aspects | Phosphotransferases | Oxidative stress | Mitochondria | Transmission electron microscopy | Rodents | Iron | Mutation | Gene expression | Index Medicus | Biological Sciences
Journal Article
Biochemistry, ISSN 0006-2960, 08/2011, Volume 50, Issue 33, pp. 7265 - 7274
Journal Article
Human Molecular Genetics, ISSN 0964-6906, 02/2005, Volume 14, Issue 4, pp. 463 - 474
Journal Article
PLoS ONE, ISSN 1932-6203, 07/2015, Volume 10, Issue 7, pp. e0132376 - e0132376
Friedreich's ataxia (FRDA), the most common inherited ataxia in the Caucasian population, is a multisystemic disease caused by a significant decrease in the... 
FRATAXIN DEFICIENCY | IRON CHAPERONE | PATHWAY | MULTIDISCIPLINARY SCIENCES | YEAST MODEL | RESISTANCE | INCREASES | EXTENDS LIFE-SPAN | MECHANISMS | EXPRESSION | OXIDATIVE STRESS-RESPONSE | Aconitate Hydratase - metabolism | Superoxide Dismutase - genetics | Glutathione - metabolism | Longevity - drug effects | Antioxidants - metabolism | Humans | Male | Aldehydes - metabolism | Drosophila Proteins - metabolism | Drosophila melanogaster - genetics | Iron-Binding Proteins - metabolism | Friedreich Ataxia - genetics | Drosophila melanogaster - metabolism | RNA Interference | Adenosine Triphosphate - metabolism | Drosophila Proteins - antagonists & inhibitors | Immunosuppressive Agents - pharmacology | Repressor Proteins - metabolism | Superoxide Dismutase - metabolism | Disease Models, Animal | Malondialdehyde - metabolism | Friedreich Ataxia - metabolism | Gene Expression | Animals, Genetically Modified | Repressor Proteins - genetics | Transcription Factors - antagonists & inhibitors | Longevity - genetics | Transcription Factors - genetics | Reverse Transcriptase Polymerase Chain Reaction | Sirolimus - pharmacology | Transcription Factors - metabolism | Motor Activity - genetics | Animals | Iron-Binding Proteins - genetics | Drosophila Proteins - genetics | Oxidative Stress - drug effects | Antioxidants | Ataxia | Rapamycin | Analysis | Drosophila | Genetic screening | Index Medicus
Journal Article
Journal Article
Journal of the Neurological Sciences, ISSN 0022-510X, 2009, Volume 287, Issue 1, pp. 111 - 118
Journal Article