Missense mutation in selenocysteine synthase causes cardio-respiratory failure and perinatal death in mice which can be compensated by selenium-independent GPX4.

GPX4 NRF2 SEPSECS Sedaghatian disease Selenoprotein

Journal

Redox biology
ISSN: 2213-2317
Titre abrégé: Redox Biol
Pays: Netherlands
ID NLM: 101605639

Informations de publication

Date de publication:
12 Nov 2021
Historique:
received: 01 10 2021
revised: 26 10 2021
accepted: 11 11 2021
pubmed: 19 11 2021
medline: 19 11 2021
entrez: 18 11 2021
Statut: aheadofprint

Résumé

Selenoproteins are a small family of proteins containing the trace element selenium in form of the rare amino acid selenocysteine (Sec), which is decoded by the UGA codon. In humans, a number of pathogenic variants in genes encoding distinct selenoproteins or selenoprotein biosynthesis factors have been identified. Pathogenic variants in selenocysteine synthase (SEPSECS), which catalyzes the last step in Sec-tRNA

Identifiants

pubmed: 34794077
pii: S2213-2317(21)00348-7
doi: 10.1016/j.redox.2021.102188
pmc: PMC8605217
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

102188

Informations de copyright

Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.

Auteurs

Noelia Fradejas-Villar (N)

Institut für Biochemie und Molekularbiologie, Universitätsklinikum Bonn, Bonn, Germany.

Wenchao Zhao (W)

Institut für Biochemie und Molekularbiologie, Universitätsklinikum Bonn, Bonn, Germany.

Uschi Reuter (U)

Institut für Biochemie und Molekularbiologie, Universitätsklinikum Bonn, Bonn, Germany.

Michael Doengi (M)

Institut für Physiologie, Universitätsklinikum Bonn, Bonn, Germany.

Irina Ingold (I)

Helmholtz Zentrum München, Institute of Metabolism and Cell Death, 85764, Neuherberg, Germany.

Simon Bohleber (S)

Institut für Biochemie und Molekularbiologie, Universitätsklinikum Bonn, Bonn, Germany.

Marcus Conrad (M)

Helmholtz Zentrum München, Institute of Metabolism and Cell Death, 85764, Neuherberg, Germany; Pirogov Russian National Research Medical University, Laboratory of Experimental Oncology, Moscow, 117997, Russia.

Ulrich Schweizer (U)

Institut für Biochemie und Molekularbiologie, Universitätsklinikum Bonn, Bonn, Germany. Electronic address: uschweiz@uni-bonn.de.

Classifications MeSH