Serine palmitoyltransferase assembles at ER-mitochondria contact sites.


Journal

Life science alliance
ISSN: 2575-1077
Titre abrégé: Life Sci Alliance
Pays: United States
ID NLM: 101728869

Informations de publication

Date de publication:
02 2022
Historique:
received: 25 10 2021
revised: 01 11 2021
accepted: 03 11 2021
entrez: 17 11 2021
pubmed: 18 11 2021
medline: 29 3 2022
Statut: epublish

Résumé

The accumulation of sphingolipid species in the cell contributes to the development of obesity and neurological disease. However, the subcellular localization of sphingolipid-synthesizing enzymes is unclear, limiting the understanding of where and how these lipids accumulate inside the cell and why they are toxic. Here, we show that SPTLC2, a subunit of the serine palmitoyltransferase (SPT) complex, catalyzing the first step in de novo sphingolipid synthesis, localizes dually to the ER and the outer mitochondrial membrane. We demonstrate that mitochondrial SPTLC2 interacts and forms a complex in trans with the ER-localized SPT subunit SPTLC1. Loss of SPTLC2 prevents the synthesis of mitochondrial sphingolipids and protects from palmitate-induced mitochondrial toxicity, a process dependent on mitochondrial ceramides. Our results reveal the in trans assembly of an enzymatic complex at an organellar membrane contact site, providing novel insight into the localization of sphingolipid synthesis and the composition and function of ER-mitochondria contact sites.

Identifiants

pubmed: 34785538
pii: 5/2/e202101278
doi: 10.26508/lsa.202101278
pmc: PMC8605320
pii:
doi:

Substances chimiques

Multienzyme Complexes 0
Serine C-Palmitoyltransferase EC 2.3.1.50

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© 2021 Aaltonen et al.

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Auteurs

Mari J Aaltonen (MJ)

Montreal Neurological Institute, McGill University, Montreal, Canada.
Department of Human Genetics, McGill University, Montreal, Canada.

Irina Alecu (I)

Department of Biochemistry, Microbiology, and Immunology, Faculty of Medicine, University of Ottawa, Ottawa, Canada.
Ottawa Institute of Systems Biology, Ottawa, Canada.

Tim König (T)

Montreal Neurological Institute, McGill University, Montreal, Canada.

Steffany Al Bennett (SA)

Department of Biochemistry, Microbiology, and Immunology, Faculty of Medicine, University of Ottawa, Ottawa, Canada.
Ottawa Institute of Systems Biology, Ottawa, Canada.

Eric A Shoubridge (EA)

Montreal Neurological Institute, McGill University, Montreal, Canada eric.shoubridge@mcgill.ca.
Department of Human Genetics, McGill University, Montreal, Canada.

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Classifications MeSH