- University of Buenos Aires, Argentina, Biology, B.S. 1983
- University of Buenos Aires, Argentina, Biology, Ph.D. 1991
- University of Massachusetts Medical Center, Biochemistry and Molecular Biology, Postdoctoral Fellow, 1991-1997
- Boston University School of Dental Medicine, Instructor, Research Assistant Professor, 1998-2006
Molecular mechanisms of glycosylation and glycoprotein processing in C. elegans.
Glycoproteins are estimated to comprise 50% of eukaryotic proteomes. In contrast to DNA, RNA and proteins, the biosynthesis of complex carbohydrates is not driven by a "template": the repertoire of glycan structures in a given cell type is primarily regulated by the activity of multiple and competitive enzymatic pathways. The resulting modifications are extremely diverse in structure: glycans have complex, branched structures and are intrinsically heterogeneous. Many glycoproteins are localized on the cell surface or secreted to the extracellular matrix, where they mediate molecular interactions critical to cell growth, inflammation, immune defense, fertilization and parasitic infection. These molecules reach their destinations by organized intracellular trafficking; it is during trafficking that they are modified by glycosylation.
The goal of our research is to gain insight into how the correct assembly of glycans is regulated and how the glycosylation status of proteins relates with their trafficking and -ultimately - with their biological activities in the context of a multicellular organism. To address these questions, we are using the nematode Caenorhabditis elegans, a genetically tractable model organism. Our research combines biochemical and genetic strategies to study the roles of the glycosylation machinery in modulating extracellular signaling pathways.
- Burnham-Marusich A.R., Snodgrass C.J., Johnson A.M., Kiyoshi C.M., Buzby S.E., Gruner M.R. and Berninsone P.M. (2012) "Metabolic Labeling of Caenorhabditis elegans Primary Embryonic Cells with Azido-Sugars as a Tool for Glycoprotein Discovery" PLoS ONE 7(11): e49020.
- Burnham-Marusich A.R. and Berninsone P.M. (2012) "Multiple proteins with essential mitochondrial functions have glycosylated isoforms" Mitochondrion 12: 423-427
- Caffaro C., Luhn K., Bakker H., Vestweber D., Samuelson J., Berninsone P. and Hirschberg C.B. (2008) A single Caenorhabditis elegans Golgi Apparatus-type transporter of UDP-Glucose, UDP-Galactose, UDP-N-Acetylglucosamine, and UDP-N-Acetylgalactosamine. Biochemistry 47: 4337- 4344
- Caffaro C.E., Hirschberg C.B. and Berninsone P.M. In Press. Functional redundancy between two C.elegans nucleotide sugar transporters with a novel transport mechanism. J.Biol.Chem.
- Berninsone, P.M. (2006) Carbohydrates and glycosylation, WormBook, ed. The C. elegans Research Community, http://www.wormbook.org
- Caffaro C.E., Hirschberg C.B. and Berninsone P.M. (2006) Independent and simultaneous translocation of two substrates by a nucleotide sugar transporter. Proc.Natl.Acad.Sci. USA 103: 16176-16181
- Burket C.T, Higgins C.E., Hull L.C., Berninsone P. and Ryder E.F. (2006) The C.elegans gene dig-1 encodes a giant member of the immunoglobulin superfamily that promotes fasciculation of neuronal processes. Dev. Biol. 299: 193-205
- Hoflich J., Berninsone P., Gobel C., Gravato-Noble M.J., Libby B.J., Darby C., Politz S.M., Hodgkin J., Hirschberg C.B. and Baumeister R. (2004) Loss of srf-3 encoded nucleotide sugar transporter activity in Caenorhabitis elegans alters surface antigenicity and prevents bacterial adherence. J.Biol.Chem. 279: 30440-30448
- Uccelletti D., O'Callaghan C., Berninsone P., Zemtseva I., Abeijon C., and Hirschberg C.B. (2004) ire-1 dependent transcriptional upregulation of a lumenal uridine diphosphatase from Caenorhabditis elegans. J.Biol.Chem. 279: 27390-27398
- Berninsone P. and Hirschberg C.B. (2003) The nematode Caenorhabditis elegans as a model to study the roles of proteoglycans. Glycoconj .J. 19: 325-330 (Review)
- Etzioni A., Sturla L., Antonellis A., Green E.D., Gersoni-Baruch R., Berninsone P.M., Hirschberg C.B. and Tonetti M. (2002) Leukocyte adhesion deficiency (LAD) type II/carbohydrate deficient glycoprotein (CDG) IIc founder effect and genotype/phenotype correlation. Am. J. Med. Genet. 110: 131-135
- Berninsone P., Hwang H-Y., Zemtseva I., Horvitz H.R. and Hirschberg C.B (2001) SQV-7, a protein involved in C. elegans epithelial invagination and early embryogenesis, transports UDP-glucuronic acid, UDP-N-acetylgalactosamine and UDP-galactose. Proc.Natl.Acad.Sci. USA 98: 3738-3743
- Berninsone P. and Hirschberg C.B. (2000) Nucleotide sugar transporters of the Golgi apparatus. Curr. Opin. Struct. Biol. 2000 10 (5): 542-547 (Review)