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Angebault 2018 Sci Signal
Additional label Labels  + , 2018-11  +
Coupling states LEAK  + , OXPHOS  +
Diseases Neurodegenerative  +
Enzyme Complex I  + , Complex II;succinate dehydrogenase  + , Complex IV;cytochrome c oxidase  +
Has abstract Communication between the endoplasmic reti
Communication between the endoplasmic reticulum (ER) and mitochondria plays a pivotal role in Ca<sup>2+</sup> signaling, energy metabolism, and cell survival. Dysfunction in this cross-talk leads to metabolic and neurodegenerative diseases. Wolfram syndrome is a fatal neurodegenerative disease caused by mutations in the ER-resident protein WFS1. Here, we showed that WFS1 formed a complex with neuronal calcium sensor 1 (NCS1) and inositol 1,4,5-trisphosphate receptor (IP<sub>3</sub>R) to promote Ca<sup>2+</sup> transfer between the ER and mitochondria. In addition, we found that NCS1 abundance was reduced in WFS1-null patient fibroblasts, which showed reduced ER-mitochondria interactions and Ca<sup>2+</sup> exchange. Moreover, in WFS1-deficient cells, NCS1 overexpression not only restored ER-mitochondria interactions and Ca<sup>2+</sup> transfer but also rescued mitochondrial dysfunction. Our results describe a key role of NCS1 in ER-mitochondria cross-talk, uncover a pathogenic mechanism for Wolfram syndrome, and potentially reveal insights into the pathogenesis of other neurodegenerative diseases.
nesis of other neurodegenerative diseases.  +
Has editor [[Plangger M]]  +
Has info [https://www.ncbi.nlm.nih.gov/pubmed/30352948 PMID: 30352948]  +
Has title Angebault C, Fauconnier J, Patergnani S, R
Angebault C, Fauconnier J, Patergnani S, Rieusset J, Danese A, Affortit CA, Jagodzinska J, Mégy C, Quiles M, Cazevieille C, Korchagina J, Bonnet-Wersinger D, Milea D, Hamel C, Pinton P, Thiry M, Lacampagne A, Delprat B, Delettre C (2018) ER-mitochondria cross-talk is regulated by the Ca2+ sensor NCS1 and is impaired in Wolfram syndrome. Sci Signal 11:eaaq1380.
Wolfram syndrome. Sci Signal 11:eaaq1380.  +
Instrument and method Oxygraph-2k  +
Mammal and model Human  +
MiP area Respiration  +
Pathways N  + , S  + , NS  +
Preparation Permeabilized cells  +
Respiration and regulation Calcium  + , Ion;substrate transport  +
Tissue and cell Fibroblast  +
Was published by MiPNetLab FR Montpellier Fauconnier J +
Was published in journal Sci Signal +
Was published in year 2018  +
Was written by Angebault C + , Fauconnier J + , Patergnani S + , Rieusset J + , Danese A + , Affortit CA + , Jagodzinska J + , Megy C + , Quiles M + , Cazevieille C + , Korchagina J + , Bonnet-Wersinger D + , Milea D + , Hamel C + , Pinton P + , Thiry M + , Lacampagne A + , Delprat B + , Delettre C +
Categories Publications
Modification date
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12:36:02, 5 November 2018  +
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