Marco Tigano

A Human Neuronal Cell Model of Endogenous TDP-43 A315T Reveals Altered Protein Dynamics and Disease-Relevant Cellular Dysfunction

This study develops a human neuronal model with the TDP-43 A315T mutation to study ALS-related protein dysfunction and therapeutic responses.

Cell-free mtDNA drives pyroptosis and inflammation in m.3243A>G mitochondrial disease models

This study shows that the m.3243A>G mutation increases cell-free mtDNA release, triggering pyroptosis and inflammation in MELAS models.

Nuclear DNA Damage Response Triggers Reorganization of Mitochondrial Nucleic Acids

This study identifies mito-blobs as nuclear DNA damage-induced mitochondrial structures that concentrate immunogenic nucleic acids and influence inflammatory gene expression through autophagic clearance.

Mutations Causative of CPEO Differentially Engage Innate Immunity Sensors

This study shows that mutations in RNASEH1 and Twinkle trigger innate immune activation through different mitochondrial nucleic acid release mechanisms, leading to paracrine inflammation in CPEO.

Peri-mitochondrial actin filaments inhibit Parkin assembly via disruption of ER-mitochondrial contact

The paper shows that peri-mitochondrial actin filaments (ADA) inhibit Parkin assembly by disrupting ER-mitochondrial contacts, delaying mitophagy after acute damage and blocking it during chronic dysfunction.

Engineering mtDNA Deletions by Reconstituting End-Joining in Human Mitochondria

This paper develops a method to engineer specific mtDNA deletions in human cells using end-joining machinery and targeted endonucleases, revealing a 75% heteroplasmy threshold for mitochondrial dysfunction.

Co-option of mitochondrial nucleic acid sensing pathways by HSV-1 UL12.5 for reactivation from latent Infection

This study shows HSV-1 UL12.5 promotes reactivation by inducing mitochondrial nucleic acid sensing, which activates innate immune pathways and supports lytic gene expression during latency exit.

Transcriptional regulation in the absence of inositol trisphosphate receptor calcium signaling

This study examines how the loss of IP3R calcium signaling affects transcriptional regulation and identifies three key adaptations in IP3R TKO cells.

Nuclear sensing of mitochondrial DNA breaks enhances immune surveillance

The paper shows that mitochondrial DNA breaks trigger a type I interferon response through nuclear sensing, enhancing immune surveillance against genotoxic stress.