The Mitochondrial Medicine group lead by Dario Brunetti, carries out translational research aimed at:
1) understanding the molecular mechanisms that link mitochondrial dysfunctions (of genetic origin or related to aging) to neuromuscular degenerative processes and metabolic disorders.
2) development of new metabolic, pharmacological and gene therapy strategies for restoring the functionality of mitochondria.
The preclinical research activity is carried out using or creating (through gene editing) both in vitro models (patient fibroblasts, iPS, NPCs, Organoids) and in vivo models (genetically modified mice and pigs).
The laboratory is located at the U.O. of Medical Genetics and Neurogenetics of the IRCCS Foundation Neurological Institute "C. Besta" and it integrates with the diagnostic and clinical activities of the other groups ensuring fruitful multidisciplinary, translational and precision medicine research (from bed to bench and back).
Currently 3 macro research areas are active:
3) Pharmacological stimulation of mitochondrial biogenesis, dynamics and mitophagy for the prevention of neuromuscular degenerative processes related to aging.
Our lab is about 350 square meters and it is equipped with the principal instruments for molecular biology including an Automated DNA sequencers ABI3130XL for Sanger's sequencing, and a MiSeq system for NGS applications; biochemistry (electrophoresis, SDS-PAGE, 1D and 2D-BNE, spectrophotometer) tissue culture rooms fully equipped for iPS cell culture and facility for viral vectors manipulation, with several light microscopes including 1 confocal microscope and a facility for radioisotope-based experiments.
PI funded projects:
2020 FRRB early career award- COD 1740526: “Precision Medicine Applied to Leigh Syndrome at different stages: development of a Neonatal metabolic supplementation and a fetal gene THERapy approach” Budget: 557.800 €
2020 Telethon spring SEED grant n. GSP20003_PAsAtaxia002: “Dysregulated Frataxin processing in the pathogenesis of PITRM1-dependent Spinocerebellar Ataxia”. Budget: 47.500 €
2020 Commissioned Research by Societè des Produits Nestlè SA: “Induction of autophagy to prevent age related diseases”; Budget: 35.000 €
2020 Bando SEED Unimi – COD. 1050: “Mitochondria and Alzheimer’s disease: focus on beta-amyloid processing by PITRM1 and mitochondrial proteases-MItoAD”.
2020 European Joint Programme on Rare Diseases (EJP RD) H2020, grant n° EJPRD20-010: “A reprogramming-based strategy for drug repositioning in patients with mitochondrial DNA-associated Leigh syndrome (MILS) “
Fondazione IRCCS Istituto Neurologico "C.Besta"
Via Temolo 4, 20126 Milano, Italy
Tel: +39 02-23942633
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