Difference between revisions of "Angebault 2018 Sci Signal"
(Created page with "{{Publication |title=Angebault C, Fauconnier J, Patergnani S, Rieusset J, Danese A, Affortit CA, Jagodzinska J, Mégy C, Quiles M, Cazevieille C, Korchagina J, Bonnet-Wersinge...") |
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|preparations=Permeabilized cells | |preparations=Permeabilized cells | ||
|enzymes=Complex I, Complex II;succinate dehydrogenase, Complex IV;cytochrome c oxidase | |enzymes=Complex I, Complex II;succinate dehydrogenase, Complex IV;cytochrome c oxidase | ||
|topics=Calcium, Ion;substrate transport | |||
|couplingstates=LEAK, OXPHOS | |couplingstates=LEAK, OXPHOS | ||
|pathways=N, S, NS | |pathways=N, S, NS | ||
Revision as of 14:36, 5 November 2018
| 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. |
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 (2018) Sci Signal
Abstract: Communication between the endoplasmic reticulum (ER) and mitochondria plays a pivotal role in Ca2+ 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 Ca2+ 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 Ca2+ exchange. Moreover, in WFS1-deficient cells, NCS1 overexpression not only restored ER-mitochondria interactions and Ca2+ 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.
• Bioblast editor: Plangger M • O2k-Network Lab: FR Montpellier Fauconnier J
Labels: MiParea: Respiration
Pathology: Neurodegenerative
Organism: Human Tissue;cell: Fibroblast Preparation: Permeabilized cells Enzyme: Complex I, Complex II;succinate dehydrogenase, Complex IV;cytochrome c oxidase Regulation: Calcium, Ion;substrate transport Coupling state: LEAK, OXPHOS Pathway: N, S, NS HRR: Oxygraph-2k
Labels, 2018-11
