E. A. Shoubridge

SLC25A46 is in contact with lysosomes and plays a role in mitochondrial cholesterol homeostasis

This study shows SLC25A46 is required for stress-induced mitochondrial hyperfusion and involved in mitochondrial cholesterol homeostasis.

ESYT1 tethers the endoplasmic reticulum to mitochondria and is required for mitochondrial lipid and calcium homeostasis

This study identifies ESYT1 as a key protein tethering ER and mitochondria at MERCs, showing its role in calcium and lipid homeostasis.

SPTLC1 variants associated with childhood onset amyotrophic lateral sclerosis produce distinct sphingolipid signatures through impaired interaction with ORMDL proteins

The paper shows that SPTLC1 variants causing childhood ALS disrupt ORMDL interactions, leading to altered sphingolipid synthesis and distinct lipid signatures, modifiable by L-serine availability.

A CHCHD10 variant causing ALS elicits an unfolded protein response through the IRE1/XBP1 pathway

This study shows that a CHCHD10 variant causes ALS by triggering an unfolded protein response via the IRE1/XBP1 pathway, leading to metabolic and stress-related changes in patient cells.

A high-density human mitochondrial proximity interaction network.

This paper creates a high-resolution human mitochondrial proximity interaction network using BioID, identifying 1465 proteins and 15626 interactions, revealing sub-compartment organization and functional modules.

LONP1 is required for maturation of a subset of mitochondrial proteins and its loss elicits an integrated stress response

This study shows that LONP1 is necessary for maturing specific mitochondrial proteins and its absence causes stress response activation and mitochondrial dysfunction.