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Dr Andrea Brancaccio

Biography

Andrea Brancaccio obtained his PhD degree in Biochemistry at the Sapienza University in Rome in 1994 characterizing recombinant myoglobins. He then moved to the Biozentrum of the University of Basel with a post-doctoral position in the laboratory of Prof. Jürgen Engel where his research interest focused on the extracellular matrix and in particular on dystroglycan. During this period, he collected the very first structural data on alpha-dystroglycan using electron microscopy on the native molecule as well as on some recombinant fragments, thus discovering an N-terminal autonomous module whose structure he subsequently contributed to solve by X-ray crystallography. During his post-doc he also investigated structurally and functionally the interaction between agrin and alpha-dystroglycan. In 1998 he moved back to Italy to join CNR at the Institute of Chemistry of Molecular Recognition (now Institute of Chemical Sciences and Technologies), where his main research interest is still the structural-functional relationships of dystroglycan.

Over the last 6 years, he has spent long periods as a visiting scientist at the School of Biochemistry of the University of Bristol (UK), where he was involved on a project on Archaea chaperonines while he kept following his main interest in dystroglycan and other extracellular matrix proteins. During these years, he was senior author of a series of structural papers on the N-terminal domain of alpha-dystroglycan (WT and mutated) which underlined the importance of its highly flexible structure in the intracellular maturation of the dystroglycan precursor. Following on this line of work, the recently characterized and ever-growing group of primary dystroglycanopathies became central to its research focus. In parallel with this experimental work, he has performed bioinformatic analyses investigating a series of evolutionary aspects of the dystroglycan protein and gene.

His work on the transmembrane beta-subunit of dystroglycan recently resulted in the characterization of a site-directed mutant of its ectodomain, C667F. This mutation was shown to cause a severe Muscle-Eye-Brain disease phenotype, and using a combined approach based on cellular biochemistry and super-resolution microscopy, Dr. Brancaccio and collaborators demonstrated that the mutated polypeptide is engulfed in the ER/Golgi (Signorino et al., Human Mutation, 2018). The murine knockin model of this mutation has been established and is under analysis in collaboration with University of Bonn. Currently, he is the recipient of a bilateral CNR-CINVESTAV grant for analyzing the interactors of beta-dystroglycan in the nucleus and has meanwhile started a new project within the Medical School of Bristol University on the potential involvement of the agrin-dystroglycan axis in cardiomyocytes regeneration.

 

Memberships

Organisations

School of Biochemistry