A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model
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Intracellular Delivery of Proteins with Cell-Penetrating Peptides for Therapeutic Uses in Human DiseaseCardiomyopathy in Friedreich ataxia: clinical findings and researchTherapeutic developments in Friedreich ataxiaTransition metals and mitochondrial metabolism in the heartAn AAV9 coding for frataxin clearly improved the symptoms and prolonged the life of Friedreich ataxia mouse modelsCell-permeable protein therapy for complex I dysfunctionMitochondria-penetrating peptides conjugated to desferrioxamine as chelators for mitochondrial labile ironProgressive mitochondrial protein lysine acetylation and heart failure in a model of Friedreich's ataxia cardiomyopathy.Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stressRationale for the development of 2-aminobenzamide histone deacetylase inhibitors as therapeutics for Friedreich ataxia.Increasing frataxin gene expression with histone deacetylase inhibitors as a therapeutic approach for Friedreich's ataxia.Management and therapy for cardiomyopathy in Friedreich's ataxia.Animal and cellular models of Friedreich ataxia.Milestones in Friedreich ataxia: more than a century and still learning.Therapeutic applications of the TAT-mediated protein transduction system for complex I deficiency and other mitochondrial diseases.Friedreich Ataxia: current status and future prospects.Therapeutic approaches for the treatment of Friedreich's ataxia.Translating HDAC inhibitors in Friedreich's ataxia.Pharmacological therapeutics in Friedreich ataxia: the present state.Replacement of the C6ORF66 assembly factor (NDUFAF4) restores complex I activity in patient cells.Apoptotic cell death and altered calcium homeostasis caused by frataxin depletion in dorsal root ganglia neurons can be prevented by BH4 domain of Bcl-xL protein.Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia.Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia.TAT-MTS-MCM fusion proteins reduce MMA levels and improve mitochondrial activity and liver function in MCM-deficient cells.Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment.Import of TAT-conjugated propionyl-CoA carboxylase using models of propionic acidemia.Low apolipoprotein A-I levels in Friedreich's ataxia and in frataxin-deficient cells: Implications for therapy.Biophysical characterisation of the recombinant human frataxin precursor.Increased Frataxin Expression Induced in Friedreich Ataxia Cells by Platinum TALE-VP64s or Platinum TALE-SunTag.Adding a temporal dimension to the study of Friedreich's ataxia: the effect of frataxin overexpression in a human cell model.Emerging therapeutics for the treatment of Friedreich’s ataxiaSynthesis of frataxin genes by direct assembly of serial deoxyoligonucleotide primers and its expression in Escherichia coli
P2860
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P2860
A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@ast
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@en
type
label
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@ast
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@en
prefLabel
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@ast
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@en
P2093
P2860
P356
P1476
A TAT-frataxin fusion protein ...... riedreich's ataxia mouse model
@en
P2093
Clifford M Babbey
Elizabeth M Martin
Jennifer Mercier
P Melanie Pride
Piyush M Vyas
Qiujuan Wang
R Mark Payne
Wendy J Tomamichel
P2860
P304
P356
10.1093/HMG/DDR554
P577
2011-11-23T00:00:00Z