G. S. Shadel

Endosomal removal and disposal of dysfunctional, immunostimulatory mitochondrial DNA

The paper investigates how dysfunctional mitochondrial DNA is removed via an endosomal pathway, showing that defects in mtDNA replication and segregation trigger a fission checkpoint leading to immune activation and eventual disposal through RAB7-containing endosomes.

Cooperative sensing of mitochondrial DNA by ZBP1 and cGAS promotes cardiotoxicity

This study shows that ZBP1 and cGAS cooperate to detect mitochondrial DNA, leading to cardiotoxicity, and that blocking this interaction protects against heart damage.

Activation of the cGAS-STING innate immune response in cells with deficient mitochondrial topoisomerase TOP1MT

This study links TOP1MT deficiency and the P193L variant to mtDNA release and cGAS-STING activation, contributing to autoimmune disease susceptibility.

ER-lysosome lipid transfer protein VPS13C/PARK23 prevents aberrant mtDNA-dependent STING signaling

The paper shows that VPS13C deficiency leads to lysosomal lipid changes and activates the cGAS/STING pathway via mtDNA accumulation and impaired STING degradation.

SARS-CoV-2 Spike Protein Impairs Endothelial Function via Downregulation of ACE2

This study shows SARS-CoV-2 Spike protein damages endothelial cells by reducing ACE2 expression and impairing mitochondrial function, leading to vascular inflammation.

Paradoxical Aortic Stiffening and Subsequent Cardiac Dysfunction in Hutchinson-Gilford Progeria Syndrome

This study shows that aortic stiffening and cardiac dysfunction in HGPS mice occur due to loss of smooth muscle function and proteoglycan accumulation, independent of sex, with potential therapeutic implications.