Our lab is interested in two main topics:
1) The pathogenic basis of hereditary spastic paraplegia
We aim to understand the mechanisms underlying axonal degeneration, a chronic process that plays an important role in several neurodegenerative diseases. We study the function of several proteins involved in hereditary spastic paraplegia, a disease characterized by degeneration of the corticospinal axons. Ultimately, we aim to a better understanding of the pathophysiology of this disease, and to unravel possible therapeutic avenues.
2) Regulatory mechanisms of mitochondrial function
Mitochondria are essential organelles involved in ATP production, Fe-S cluster biogenesis, calcium buffering, and several metabolic biosynthetic pathways. Mitochondrial homeostasis depends on regulated biogenesis and efficient quality control mechanisms, and is key to cell survival. Dysfunctional mitochondria can trigger apoptotic cell death, and play an important role in the pathogenesis of several diseases, including hereditary spastic paraplegia. We are investigating post-transcriptional mechanisms of regulation of mitochondrial biogenesis, as well as quality control pathways that are crucial to maintain the organelle function.