In vivo maturation of human frataxin (Q24304209)

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30 December 2019

title

In vivo maturation of human frataxin (English)

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30 December 2019

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GOA release 2020-03-11

author

Ivano Condò

1

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30 December 2019

Natascia Ventura

2

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30 December 2019

Florence Malisan

3

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30 December 2019

5

Barbara Tomassini

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30 December 2019

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author name string

Alessandra Rufini

4

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30 December 2019

Roberto Testi

6

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30 December 2019

language of work or name

English

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publication date

27 April 2007

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30 December 2019

published in

Human Molecular Genetics

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volume

16

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issue

13

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30 December 2019

page(s)

1534-1540

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Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

30 December 2019

cites work

1 reference

Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes

21 January 2018

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Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis

21 January 2018

1 reference

Iron-sulfur protein maturation in human cells: evidence for a function of frataxin

21 January 2018

1 reference

Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia

21 January 2018

1 reference

Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

21 January 2018

1 reference

Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin

21 January 2018

1 reference

Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia

21 January 2018

1 reference

Frataxin activates mitochondrial energy conversion and oxidative phosphorylation

21 January 2018

1 reference

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

21 January 2018

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Frataxin expression rescues mitochondrial dysfunctions in FRDA cells

21 January 2018

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A pool of extramitochondrial frataxin that promotes cell survival

21 January 2018

1 reference

Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation

21 January 2018

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Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia

21 January 2018

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The structure and function of frataxin

21 January 2018

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Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

21 January 2018

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Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates

21 January 2018

1 reference

Mitochondrial processing peptidases

21 January 2018

1 reference

Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis

21 January 2018

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Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase.

21 January 2018

1 reference

Maturation of frataxin within mammalian and yeast mitochondria: one-step processing by matrix processing peptidase

21 January 2018

1 reference

Crystal structures of mitochondrial processing peptidase reveal the mode for specific cleavage of import signal sequences

21 January 2018

1 reference

Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide

21 January 2018

1 reference

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

21 January 2018

1 reference

Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis

21 January 2018

1 reference

Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins

21 January 2018

1 reference

Reversible redox-dependent modulation of mitochondrial aconitase and proteolytic activity during in vivo cardiac ischemia/reperfusion

21 January 2018

1 reference

Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin

21 January 2018

1 reference

Crystal structure of human frataxin

21 January 2018

1 reference

N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins

21 January 2018

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Iron-induced oligomerization of yeast frataxin homologue Yfh1 is dispensable in vivo

21 January 2018

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Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

21 January 2018

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Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region

21 January 2018

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7 January 2021

Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells

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7 January 2021

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7 January 2021

Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription

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7 January 2021

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

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7 January 2021

Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia

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7 January 2021