Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins.
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Multitasking in the mitochondrion by the ATP-dependent Lon proteaseMitochondrial Lon protease in human disease and aging: Including an etiologic classification of Lon-related diseases and disordersOverexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levelsNeurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia.MtSNPscore: a combined evidence approach for assessing cumulative impact of mitochondrial variations in disease.Posttranslational stability of the heme biosynthetic enzyme ferrochelatase is dependent on iron availability and intact iron-sulfur cluster assembly machinery.ISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo.Potential therapeutic benefits of strategies directed to mitochondria.Biological roles of the Podospora anserina mitochondrial Lon protease and the importance of its N-domain.The diabetes drug target MitoNEET governs a novel trafficking pathway to rebuild an Fe-S cluster into cytosolic aconitase/iron regulatory protein 1Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.Upregulation of the mitochondrial Lon Protease allows adaptation to acute oxidative stress but dysregulation is associated with chronic stress, disease, and aging.Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer.Down-regulating overexpressed human Lon in cervical cancer suppresses cell proliferation and bioenergetics.HSC20 interacts with frataxin and is involved in iron-sulfur cluster biogenesis and iron homeostasis.Mitochondrial Lon regulates apoptosis through the association with Hsp60-mtHsp70 complex.Identification of potential mitochondrial CLPXP protease interactors and substrates suggests its central role in energy metabolism.Tissue specificity of a human mitochondrial disease: differentiation-enhanced mis-splicing of the Fe-S scaffold gene ISCU renders patient cells more sensitive to oxidative stress in ISCU myopathy.Mitochondrial ClpP activity is required for cisplatin resistance in human cells.Overexpression of Lon contributes to survival and aggressive phenotype of cancer cells through mitochondrial complex I-mediated generation of reactive oxygen species.Pharmacological approaches to restore mitochondrial function.Diverse functions of mitochondrial AAA+ proteins: protein activation, disaggregation, and degradation.Impaired TCA cycle flux in mitochondria in skeletal muscle from type 2 diabetic subjects: marker or maker of the diabetic phenotype?Down-regulation of the mitochondrial matrix peptidase ClpP in muscle cells causes mitochondrial dysfunction and decreases cell proliferation.Autophagy compensates impaired energy metabolism in CLPXP-deficient Podospora anserina strains and extends healthspan.Diphenylarsinic acid promotes degradation of glutaminase C by mitochondrial Lon protease.Friedreich ataxia-induced pluripotent stem cell-derived neurons show a cellular phenotype that is corrected by a benzamide HDAC inhibitor.Iron regulatory protein deficiency compromises mitochondrial function in murine embryonic fibroblasts.
P2860
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P2860
Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh-hant
name
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@en
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@nl
type
label
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@en
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@nl
prefLabel
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@en
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@nl
P2093
P2860
P1433
P1476
Frataxin deficiency causes upr ...... f mitochondrial Fe-S proteins.
@en
P2093
Blanche Guillon
Cécile Bouton
Hélène Puccio
Jean-Claude Drapier
Marie Wattenhofer-Donzé
Stéphane Schmucker
P2860
P304
P356
10.1111/J.1742-4658.2008.06847.X
P407
P577
2008-01-12T00:00:00Z