membrane trafficking, RNA metabolism, Duchenne Muscular Dystrophy 

Group leader : Maria Grazia Di Certo
Keywords : membrane trafficking, RNA metabolism, Duchenne Muscular Dystrophy 
Topics : Neurobiology, Muscle physiology and pathology

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Our main research interest is to understand how RNA-related proteins impact cellular/tissue homeostasis in health and disease.
Ongoing studies aim to assess the intriguing interplay between RNA metabolism and membrane trafficking regulating gene expression at subcellular level. We use a multi-disciplinary approach involving cell imaging and biochemical methods to isolate and characterize RNA-protein complexes associated to different organelles and membrane subdomains. We have a long-standing interest in neuromuscular pathologies, such as Duchenne Muscular Dystrophy (DMD) and Spinal Muscular Atrophy (SMA), to provide new insight into pathogenic mechanisms as well as to develop therapeutic strategies

 

Key Pubblications

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• Pisani C, Strimpakos G, Gabanella F, Di Certo MG, Onori A, Severini C, Luvisetto S, Farioli-Vecchioli S, Carrozzo I, Esposito A, Canu T, Mattei E, Corbi N, Passananti C. Utrophin up-regulation by artificial transcription factors induces muscle rescue and impacts the neuromuscular junction in mdx mice. Biochim Biophys Acta Mol Basis Dis. 2018 Apr; 1864:1172-1182.

• Gabanella F, Pisani C, Borreca A, Farioli-Vecchioli S, Ciotti MT, Ingegnere T, Onori A, Ammassari-Teule M, Corbi N, Canu N, Monaco L, Passananti C, Di Certo MG. SMN affects membrane remodelling and anchoring of the protein synthesis machinery. J Cell Sci. 2016 Feb 15;129(4):804-16.

• Di Certo MG, Corbi N, Strimpakos G, Onori A, Luvisetto S, Severini C, Guglielmotti A, Batassa EM, Pisani C, Floridi A, Benassi B, Fanciulli M, Magrelli A, Mattei E, Passananti C. The artificial gene Jazz, a transcriptional regulator of utrophin, corrects the dystrophic pathology in mdx mice. Hum Mol Genet. 2010 Mar 1;19(5):752-60.

• Di Certo MG, Batassa EM, Casella I, Serafino A, Floridi A, Passananti C, Molinari P, Mattei E.Delayed internalization and lack of recycling in a beta2-adrenergic receptor fused to the G protein alpha-subunit. BMC Cell Biol. 2008 Oct 7;9: 56. doi: 10.1186/1471-2121-9-56

• Di Certo MG, Corbi N, Bruno T, Iezzi S, De Nicola F, Desantis A, Ciotti MT, Mattei E, Floridi A, Fanciulli M, Passananti C. NRAGE associates with the anti-apoptotic factor Che-1 and regulates its degradation to induce cell death. J Cell Sci. 2007 Jun 1;120(Pt 11):1852-8. Epub 2007 May 8.

Research Group

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Programs & Resoucers

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2018-2021 Lazio Innova Project 15286 Passananti (PI). Title: “Geni artificiali come strategia terapeutica per la Distrofia muscolare di Duchenne”. (Artificial genes as therapeutic strategy for Duchenne Muscular Dystrophy). (Role: Co-Investigator).

2017-2018 InterOmics Project 19699 Passananti (PI). Project title: “Cell-based Omics approaches to identify pathways implicated in Duchenne Muscular Dystrophy by the use of therapeutic artificial genes”. (Role: Co-Investigator).

2017-2019 Scientific Cooperation Agreement CNR-CONICET 11985 Corbi (PI). Project title: “Mechanisms of transcription in memory persistence. Role of NF-kB and Che-1” (Role: Co-Investigator).

2014-2017 Telethon project GGP14073 Corbi (PI). Project title: “Innovative therapeutic strategy for Duchenne Muscular Dystrophy by AAV mediated delivery of artificial transcription factor genes.” (Role: Co-Investigator).

2013-2015 Association Française contre les Myopathies (AFM) grant number 15586 Monaco (PI). Project title: “PDE inhibitors as possible pharmacological therapy for muscular dystrophy.” (Role: Co-Investigator).

2011/2012 Telethon Project GGP10094 Mattei (PI). Project title: “Experimental gene therapy of Duchenne Muscular Dystrophy by artificial transcription factors upregulating the dystrophin-related Utrophin.” (Role: Co-Investigator).

2010/2013 AIRC Project IG2009 Passananti (PI). Project title: “Anti-apoptotic role of Che-1/Hax1 interaction in sever congenital neutropenia (SCN), a preleukemic condition.” (Role: Co-Investigator).

2007-2009 Telethon project GGP07177 Mattei (PI). Project title: “Animal models to validate the use of new small synthetic transcription factors in the gene therapy of muscle dystrophies.” (Role: Co-Investigator).