New roles of LPGAT1: From mitochondrial import of phosphatidylglycerol to MEGDEL disease

Vera Filipa Monteiro-Cardoso; Francesca Giordano

doi:10.1016/j.celrep.2023.113376

New roles of LPGAT1: From mitochondrial import of phosphatidylglycerol to MEGDEL disease

Cell Rep. 2023 Nov 28;42(11):113376. doi: 10.1016/j.celrep.2023.113376. Epub 2023 Nov 2.

Authors

Vera Filipa Monteiro-Cardoso¹, Francesca Giordano²

Affiliations

¹ Institute for Integrative Biology of the Cell (I2BC), CEA, CNRS, Université Paris-Saclay, Gif-sur-Yvette cedex 91198, France; Inserm U1280, Gif-sur-Yvette cedex 91198, France.
² Institute for Integrative Biology of the Cell (I2BC), CEA, CNRS, Université Paris-Saclay, Gif-sur-Yvette cedex 91198, France; Inserm U1280, Gif-sur-Yvette cedex 91198, France. Electronic address: francesca.giordano@i2bc.paris-saclay.fr.

PMID: 37917588
DOI: 10.1016/j.celrep.2023.113376

Abstract

Dysregulation of mitochondrial lipidome is associated with several human pathologies. Sun et al.¹ show that LPGAT1 cooperates with TIMM14 to regulate phosphatidylglycerol transport from the endoplasmic reticulum to the mitochondria, and uncover the involvement of LPGAT1 deficiency in MEGDEL syndrome.

Publication types

Comment

MeSH terms

Endoplasmic Reticulum / metabolism
Humans
Mitochondria* / pathology
Phosphatidylglycerols* / metabolism

Substances

Phosphatidylglycerols