about
Cardiomyopathy in Friedreich ataxia: clinical findings and researchIron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster deliveryThe role of mitochondria in cellular iron-sulfur protein biogenesis: mechanisms, connected processes, and diseasesMechanisms of mammalian iron homeostasisFriedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly ComplexFunctional reconstitution of mitochondrial Fe/S cluster synthesis on Isu1 reveals the involvement of ferredoxinSpecialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fe-4S] proteins.Inactivation of mitochondrial aspartate aminotransferase contributes to the respiratory deficit of yeast frataxin-deficient cells.The physiological basis of therapies for cerebellar ataxiasOverexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levelsGenetic variation in iron metabolism is associated with neuropathic pain and pain severity in HIV-infected patients on antiretroviral therapy.Mitochondrial optic neuropathies - disease mechanisms and therapeutic strategies.The optic nerve: a "mito-window" on mitochondrial neurodegenerationDefective mitochondrial mRNA maturation is associated with spastic ataxiaMolecular strategies of microbial iron assimilation: from high-affinity complexes to cofactor assembly systems.Mitochondrial NADH kinase, Pos5p, is required for efficient iron-sulfur cluster biogenesis in Saccharomyces cerevisiae.Mitochondrial Fe-S cluster biogenesis, frataxin and the modulation of susceptibility to drug-induced cardiomyopathy.Overlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold proteinNovel frataxin isoforms may contribute to the pathological mechanism of Friedreich ataxiaThe L-cysteine desulfurase NFS1 is localized in the cytosol where it provides the sulfur for molybdenum cofactor biosynthesis in humansCo-precipitation of phosphate and iron limits mitochondrial phosphate availability in Saccharomyces cerevisiae lacking the yeast frataxin homologue (YFH1).Fe-S cluster biogenesis in isolated mammalian mitochondria: coordinated use of persulfide sulfur and iron and requirements for GTP, NADH, and ATP.Aprataxin localizes to mitochondria and preserves mitochondrial functionCardiomyopathy of Friedreich's ataxia: use of mouse models to understand human disease and guide therapeutic development.Frataxin participates to the hypoxia-induced response in tumorsDisruption of the ATP-binding cassette B7 (ABTM-1/ABCB7) induces oxidative stress and premature cell death in Caenorhabditis elegansConsensus paper: management of degenerative cerebellar disorders.Gene expression profiling of mitochondrial oxidative phosphorylation (OXPHOS) complex I in Friedreich ataxia (FRDA) patients.Tangled web of interactions among proteins involved in iron-sulfur cluster assembly as unraveled by NMR, SAXS, chemical crosslinking, and functional studies.Isd11p protein activates the mitochondrial cysteine desulfurase Nfs1p protein.The role of induced pluripotent stem cells in regenerative medicine: neurodegenerative diseases.A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse modelBiogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.Frataxin Is Localized to Both the Chloroplast and Mitochondrion and Is Involved in Chloroplast Fe-S Protein Function in ArabidopsisA Yeast/Drosophila Screen to Identify New Compounds Overcoming Frataxin DeficiencyComing into view: eukaryotic iron chaperones and intracellular iron delivery.Disorders of the optic nerve in mitochondrial cytopathies: new ideas on pathogenesis and therapeutic targets.Activation of the HIF prolyl hydroxylase by the iron chaperones PCBP1 and PCBP2.Key players and their role during mitochondrial iron-sulfur cluster biosynthesisInterferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model.
P2860
Q26827933-D64CD789-E4A6-4D14-866E-9F2E1AE9BFB2Q26850111-A86EBAF3-A80A-4617-B2F6-82EE1A5BA646Q26865882-C6F5F11F-AEF6-400F-B191-F0953EC40E65Q27010510-C3151BB8-A7EC-4A13-BE4E-BF207F0B850DQ27670432-D02A1330-921D-4EDD-B99D-FCEB24365FB5Q27695962-88488F78-0E44-4478-8BA7-125958FCD95DQ27931818-EF391BE5-C9D6-43BD-A048-5A186942E67BQ27939892-03B10AE3-F0E5-4951-9E09-28D4F096D4F4Q28075917-032DFD91-EE6E-45DA-BBFD-DF224EE09ABCQ28479044-B0C13618-8CEC-4346-9F58-79AA0891A388Q34075188-EDCDD6C5-ABEF-4678-9533-EC0C385225F4Q34152110-1CF3EFF8-07E4-4AB4-B550-246A44477D85Q34298350-5E576241-2920-4002-AB67-5B5288682B5AQ34300940-A38B1946-3424-44FF-833E-91CC6FA6826AQ34314306-B1A05E28-E38B-4450-9065-C666DA94A7E0Q34385965-C511E676-871E-4BFB-9503-FE8A70AF839AQ34424872-D924DF49-0F9E-4B74-960D-8FCCC28BECE5Q34430900-C637A107-AEE6-4CF7-8E8E-7EDF182BC886Q34452330-465DAD97-9647-4D36-B468-AF0A2B48DC0BQ34674294-C12AC6F9-8F2E-40C6-97E3-36B54D14AAD4Q34675842-9FBFA838-640C-4A17-93BA-2EE95ACC538FQ34801755-DED77DAD-F8A0-42B7-BC6F-92F330A576F2Q34937780-031E0E0A-D20C-4ABE-A529-ED2E6A892499Q34989407-59958FF7-C2B1-48AC-B840-05F81B7FAC55Q35006031-12C3FE3F-ABB8-438A-B946-D6CD84BC783EQ35065169-0AC75E12-6CDF-40A1-8457-682F2223C877Q35129909-ED542DBA-80FF-44CC-BB48-1B43B0A2ED0AQ35140340-75769D08-5015-47B4-BE31-7BDEA9A430ABQ35327865-088B2840-4C35-485B-9616-3D87EA31C1C2Q35515660-E2193038-6395-4349-885F-05FDF199445EQ35558500-717AFE53-95C1-4083-9F94-1D4BA5BCF126Q35770976-9F725AAA-CC4D-4DA9-A523-07D98AC5281AQ35794543-7F6C5A0C-BDD1-4276-AD0D-E0CEA9DD6B88Q35827105-513FEE85-B4EE-402F-977C-858356C83CA8Q35828968-F15F7F2F-9562-4B36-BDD6-2BED65EDDF20Q35922222-3DEC043F-A26A-4932-B8CF-7FE4822130B7Q35928805-1936DDB5-C9F1-48BE-AAB0-310EAD4D4167Q35993412-CC78FE9F-8639-4B84-8185-17909BA09D29Q36005200-8690822E-3551-4DAD-A919-3CF52305FA7BQ36026884-A50856CA-0630-4779-B200-3F785099E6AC
P2860
description
2010 nî lūn-bûn
@nan
2010 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Frataxin and mitochondrial FeS cluster biogenesis.
@ast
Frataxin and mitochondrial FeS cluster biogenesis.
@en
Frataxin and mitochondrial FeS cluster biogenesis.
@nl
type
label
Frataxin and mitochondrial FeS cluster biogenesis.
@ast
Frataxin and mitochondrial FeS cluster biogenesis.
@en
Frataxin and mitochondrial FeS cluster biogenesis.
@nl
prefLabel
Frataxin and mitochondrial FeS cluster biogenesis.
@ast
Frataxin and mitochondrial FeS cluster biogenesis.
@en
Frataxin and mitochondrial FeS cluster biogenesis.
@nl
P2093
P2860
P356
P1476
Frataxin and mitochondrial FeS cluster biogenesis
@en
P2093
Andrew Dancis
Debkumar Pain
Emmanuel Lesuisse
P2860
P304
26737-26743
P356
10.1074/JBC.R110.118679
P407
P577
2010-06-03T00:00:00Z