Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts.
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Towards a unifying, systems biology understanding of large-scale cellular death and destruction caused by poorly liganded iron: Parkinson's, Huntington's, Alzheimer's, prions, bactericides, chemical toxicology and others as examplesHyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxiaStructural characterization of metal binding to a cold-adapted frataxinThe Friedreich's ataxia protein frataxin modulates DNA base excision repair in prokaryotes and mammalsIron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseasesThe first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxiaFrataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissuesDyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.The in vivo mitochondrial two-step maturation of human frataxin.Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screenMitochondria-Derived Damage-Associated Molecular Patterns in Neurodegeneration.Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Intracellular iron transport and storage: from molecular mechanisms to health implications.Defects in mitochondrial axonal transport and membrane potential without increased reactive oxygen species production in a Drosophila model of Friedreich ataxiaFrataxin participates to the hypoxia-induced response in tumorsEssential role of the iron-sulfur cluster binding domain of the primase regulatory subunit Pri2 in DNA replication initiation.A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse modelFrataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia.Advancements in the pathophysiology of Friedreich's Ataxia and new prospects for treatments.Mitochondrial localization of human frataxin is necessary but processing is not for rescuing frataxin deficiency in Trypanosoma brucei.Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers.Coenzyme A corrects pathological defects in human neurons of PANK2-associated neurodegeneration.Prenatal iron deficiency and monoamine oxidase A (MAOA) polymorphisms: combined risk for later cognitive performance in rhesus monkeys.Mössbauer study and modeling of iron import and trafficking in human jurkat cells.Mitochondrial pathophysiology in Friedreich's ataxia.Animal and cellular models of Friedreich ataxia.Diabetes in Friedreich ataxia.Aconitase post-translational modification as a key in linkage between Krebs cycle, iron homeostasis, redox signaling, and metabolism of reactive oxygen species.Oxidative stress in inherited mitochondrial diseases.Compound heterozygous FXN mutations and clinical outcome in friedreich ataxia.Frataxin deficiency induces Schwann cell inflammation and death.Two Plant-Derived Aporphinoid Alkaloids Exert Their Antifungal Activity by Disrupting Mitochondrial Iron-Sulfur Cluster Biosynthesis.Frataxin depletion in yeast triggers up-regulation of iron transport systems before affecting iron-sulfur enzyme activitiesTime-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia.Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans.Early cerebellar deficits in mitochondrial biogenesis and respiratory chain complexes in the KIKO mouse model of Friedreich ataxia.Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia.Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in a Drosophila Model of Friedreich's Ataxia.Variations of frataxin protein levels in normal individuals.
P2860
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P2860
Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
Frataxin knockdown causes loss ...... induction of heme transcripts.
@en
type
label
Frataxin knockdown causes loss ...... induction of heme transcripts.
@en
prefLabel
Frataxin knockdown causes loss ...... induction of heme transcripts.
@en
P2860
P1476
Frataxin knockdown causes loss ...... induction of heme transcripts.
@en
P2093
Gino Cortopassi
P2860
P304
P356
10.1016/J.ABB.2006.09.010
P407
P577
2006-10-04T00:00:00Z