Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis.

Activating Transcription Factor 4 / metabolism Animals Caenorhabditis elegans / metabolism Female Friedreich Ataxia / metabolism HEK293 Cells Humans Hypoxia / metabolism Iron / metabolism Iron Regulatory Protein 2 / metabolism Iron-Binding Proteins / metabolism Iron-Sulfur Proteins / metabolism K562 Cells Male Mice Mice, Knockout Mitochondria / metabolism Mitochondrial Proteins / metabolism NF-E2-Related Factor 2 / metabolism Oxidative Stress Saccharomyces cerevisiae / metabolism Sulfur / metabolism Frataxin

Journal

Cell
ISSN: 1097-4172
Titre abrégé: Cell
Pays: United States
ID NLM: 0413066

Informations de publication

Date de publication:
30 05 2019
Historique:
received: 25 06 2018
revised: 11 02 2019
accepted: 22 03 2019
pubmed: 30 4 2019
medline: 25 3 2020
entrez: 30 4 2019
Statut: ppublish

Résumé

Friedreich's ataxia (FRDA) is a devastating, multisystemic disorder caused by recessive mutations in the mitochondrial protein frataxin (FXN). FXN participates in the biosynthesis of Fe-S clusters and is considered to be essential for viability. Here we report that when grown in 1% ambient O

Identifiants

DOI: 10.1016/j.cell.2019.03.045 PMID: 31031004 PMC: PMC6911770
pubmed: 31031004
pii: S0092-8674(19)30346-0
doi: 10.1016/j.cell.2019.03.045
pmc: PMC6911770
mid: NIHMS1060410
pii:
doi:

Substances chimiques

ATF4 protein, human 0
Iron-Binding Proteins 0
Iron-Sulfur Proteins 0
Mitochondrial Proteins 0
NF-E2-Related Factor 2 0
NFE2L2 protein, human 0
Activating Transcription Factor 4 145891-90-3
Sulfur 70FD1KFU70
Iron E1UOL152H7
Iron Regulatory Protein 2 EC 4.2.1.3

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1507-1521.e16

Subventions

Organisme : NIA NIH HHS
ID : R01 AG016636
Pays : United States
Organisme : NIGMS NIH HHS
ID : R01 GM096100
Pays : United States
Organisme : NIGMS NIH HHS
ID : R35 GM122455
Pays : United States
Organisme : Howard Hughes Medical Institute
Pays : United States

Informations de copyright

Copyright © 2019 Elsevier Inc. All rights reserved.

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Auteurs

Tslil Ast (T)

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

Joshua D Meisel (JD)

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

Shachin Patra (S)

Department of Chemistry, Texas A&M University, College Station, TX 77843, USA.

Hong Wang (H)

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

Robert M H Grange (RMH)

Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.

Sharon H Kim (SH)

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

Sarah E Calvo (SE)

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

Lauren L Orefice (LL)

Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

Fumiaki Nagashima (F)

Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.

Fumito Ichinose (F)

Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.

Warren M Zapol (WM)

Department of Anesthesia, Critical Care, and Pain Medicine, Massachusetts General Hospital, Boston, MA 02114, USA.

Gary Ruvkun (G)

Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA.

David P Barondeau (DP)

Department of Chemistry, Texas A&M University, College Station, TX 77843, USA.

Vamsi K Mootha (VK)

Broad Institute, Cambridge, MA 02142, USA; Howard Hughes Medical Institute, Howard Hughes Medical Institute, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Molecular Biology, Massachusetts General Hospital, Boston, MA 02114, USA; Harvard Medical School, Boston, MA 02115, USA. Electronic address: vamsi@hms.harvard.edu.

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Classifications MeSH

questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Facteurs de transcription › Facteurs de transcription à motif basique et à glissière à leucines › Facteurs de transcription ATF › Facteur de transcription ATF-4 › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Eucaryotes › Animaux › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Eucaryotes › Animaux › Invertébrés › Helminthes › Nematoda › Chromadorea › Rhabditida › Rhabditoidea › Caenorhabditis › Caenorhabditis elegans › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Maladies mitochondriales › Ataxie de Friedreich › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Cellules › Cellules épithéliales › Cellules HEK293 › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › États, signes et symptômes pathologiques › Signes et symptômes › Signes et symptômes respiratoires › Hypoxie › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Produits chimiques inorganiques › Métaux › Métaux lourds › Fer › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Nucléoprotéines › Protéines de liaison à l'ARN › Protéine-2 de régulation du fer › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Métalloprotéines › Protéines de liaison au fer › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Métalloprotéines › Protéines de liaison au fer › Protéines à fer non hème › Ferrosulfoprotéines › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Cellules › Cellules érythroïdes › Cellules K562 › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Souris transgéniques › Souris knockout › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Cellules › Structures cellulaires › Fractions subcellulaires › Mitochondries › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines mitochondriales › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Facteurs de transcription › Facteurs de transcription à motif basique et à glissière à leucines › Facteur-2 apparenté à NF-E2 › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Phénomènes physiologiques › Stress physiologique › Stress oxydatif › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Eucaryotes › Champignons › Levures › Saccharomyces › Saccharomyces cerevisiae › Hypoxia Rescues Frataxin Loss by Restoring...
questionsmedicales.fr › Produits chimiques inorganiques › Éléments › Chalcogènes › Soufre › Hypoxia Rescues Frataxin Loss by Restoring...