ER-mitochondria cross-talk is regulated by the Ca(2+) sensor NCS1 and is impaired in Wolfram syndrome.

[en] Communication between the endoplasmic reticulum (ER) and mitochondria plays a pivotal role in Ca(2+) 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 (IP3R) to promote Ca(2+) 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(2+) exchange. Moreover, in WFS1-deficient cells, NCS1 overexpression not only restored ER-mitochondria interactions and Ca(2+) 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.

Disciplines :

Biochemistry, biophysics & molecular biology

Author, co-author :

Angebault, Claire

Fauconnier, Jeremy

Patergnani, Simone

Rieusset, Jennifer

Danese, Alberto

Affortit, Corentin A.

Jagodzinska, Jolanta

Megy, Camille

Quiles, Melanie

Cazevieille, Chantal

More authors (9 more)

Language :

English

Title :

ER-mitochondria cross-talk is regulated by the Ca(2+) sensor NCS1 and is impaired in Wolfram syndrome.

Publication date :

2018

Journal title :

Science Signaling

ISSN :

1945-0877

eISSN :

1937-9145

Publisher :

American Association for the Advancement of Science, Washington, United States - District of Columbia

Volume :

Issue :

553

Peer reviewed :

Peer Reviewed verified by ORBi

Commentary :

Available on ORBi :

since 16 November 2018

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