Is aberrant N-glucosylation relevant to recognise anti-MOG antibodies in Rett syndrome?

  • Feliciana Real-Fernández Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Chemistry “Ugo Schiff”, University of Florence, Sesto Fiorentino, Italy
  • Giulia Pacini Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Neurosciences, Psychology, Drug Research and Child Health - Section of Pharmaceutical Sciences and Nutraceutics, University of Florence, Sesto Fiorentino, Italy
  • Francesca Nuti Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Chemistry “Ugo Schiff”, University of Florence, Sesto Fiorentino, Italy
  • Giulia Conciarelli Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Neurosciences, Psychology, Drug Research and Child Health - Section of Pharmaceutical Sciences and Nutraceutics, University of Florence, Sesto Fiorentino, Italy
  • Claudio De Felice Neonatal Intensive Care Unit, University Hospital, Azienda Ospedaliera Universitaria Senese, Siena, Italy
  • Joussef Hayek Child Neuropsychiatry Unit, University Hospital, Azienda Ospedaliera Universitaria Senese, Siena, Italy
  • Paolo Rovero Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Neurosciences, Psychology, Drug Research and Child Health - Section of Pharmaceutical Sciences and Nutraceutics, University of Florence, Sesto Fiorentino, Italy
  • Anna Maria Papini Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Chemistry “Ugo Schiff”, University of Florence, Sesto Fiorentino, Italy
Keywords: Myelin oligodendrocyte glycoprotein, Rett syndrome, antibody detection, ELISA

Abstract

Antibodies against myelin oligodendrocyte glycoprotein (MOG) are associated to several disorders, and their occurrence in patients presenting an acquired demyelinating disease affects a higher proportion of paediatric subjects, as compared to adults. Despite heterogeneity in clinical presentation, few connexions have been reported between the progressive neurodevelopmental disorder affecting child’s brain development and cognitive ability, i.e. Rett syndrome (RTT), and a demyelination process. In order to identify the possible target of humoral autoimmune response in RTT patients, we set-up a home-made solid-phase ELISA, using the recombinant extracellular portion of human MOG(1-117) as an antigen. The screening to evaluate anti-MOG antibodies in RTT patient sera, compared to other relative non-RTT pervasive developmental disorders (non-RTT PDD), including mainly autism, and a healthy control group gave uncertain results. In fact, Student t-test and Mann-Whitney unpaired t test showed that differences in both IgG and IgM antibody titres between the different patient populations, were not statistically significant. We can conclude that the absence of anti-MOG antibody recognition in RTT has possibly to be ascribed to a different relevant protein folding and/or to the lack of a relevant aberrant post-translational modification, such as N-glucosylation, that we previously demonstrated, for the first time, fundamental to recognize antibodies in RTT.

Author Biography

Anna Maria Papini, Interdepartmental Laboratory of Peptide and Protein Chemistry and Biology, Department of Chemistry “Ugo Schiff”, University of Florence, Sesto Fiorentino, Italy

Anna Maria Papini is Professor of bioorganic chemistry and coordinator of PeptLab (www.peptlab.eu) who started at  Università di Firenze after her PhD supervised by Luis Moroder (Max Planck Institute for Biochemistry, Martinsried, Germany) and Gianfranco Rapi, Department of Organic Chemistry,  Università di Firenze. She obtained Leonidas Zervas Award 2018 for outstanding contribution to peptide science and since 2017 she coordinates the scientific activities of PeptFarm "Laboratorio congiunto PeptLab-Università di Firenze – Fabbrica Italiana Sintetici" to develop large-scale synthesis of peptide-drugs. She chaired the Gordon Research Conference on Peptides in Ventura (CA, USA) in 2016 and was nominated chair of 14th International Peptide Symposium that she will organise at "Palazzo dei Congressi" in Florence in 2022.

Published
2019-10-01
How to Cite
Real-Fernández, F., Pacini, G., Nuti, F., Conciarelli, G., De Felice, C., Hayek, J., Rovero, P., & Papini, A. M. (2019). Is aberrant N-glucosylation relevant to recognise anti-MOG antibodies in Rett syndrome?. Substantia, 3(2), 19-25. https://doi.org/10.13128/Substantia-632
Section
Research Articles