Recognition Memory in Noonan Syndrome
Noonan syndrome (NS) and the clinically related NS with multiple lentiginous (NMLS) are genetic conditions characterized by upregulated RAS mitogen activated protein kinase (RAS–MAPK) signaling, which is known to impact hippocampus-dependent memory formation and consolidation. The aim of the present...
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MDPI AG
2021-01-01
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| Online Access: | https://www.mdpi.com/2076-3425/11/2/169 |
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| author | Floriana Costanzo Paolo Alfieri Cristina Caciolo Paola Bergonzini Francesca Perrino Giuseppe Zampino Chiara Leoni Deny Menghini Maria Cristina Digilio Marco Tartaglia Stefano Vicari Giovanni Augusto Carlesimo |
| author_facet | Floriana Costanzo Paolo Alfieri Cristina Caciolo Paola Bergonzini Francesca Perrino Giuseppe Zampino Chiara Leoni Deny Menghini Maria Cristina Digilio Marco Tartaglia Stefano Vicari Giovanni Augusto Carlesimo |
| author_sort | Floriana Costanzo |
| collection | DOAJ |
| description | Noonan syndrome (NS) and the clinically related NS with multiple lentiginous (NMLS) are genetic conditions characterized by upregulated RAS mitogen activated protein kinase (RAS–MAPK) signaling, which is known to impact hippocampus-dependent memory formation and consolidation. The aim of the present study was to provide a detailed characterization of the recognition memory of children and adolescents with NS/NMLS. We compared 18 children and adolescents affected by NS and NMLS with 22 typically developing (TD) children, matched for chronological age and non-verbal Intelligence Quotient (IQ), in two different experimental paradigms, to assess familiarity and recollection: a Process Dissociation Procedure (PDP) and a Task Dissociation Procedure (TDP). Differences in verbal skills between groups, as well as chronological age, were considered in the analysis. Participants with NS and NSML showed reduced recollection in the PDP and impaired associative recognition in the TDP, compared to controls. These results indicate poor recollection in the recognition memory of participants with NS and NSML, which cannot be explained by intellectual disability or language deficits. These results provide evidence of the role of mutations impacting RAS–MAPK signaling in the disruption of hippocampal memory formation and consolidation. |
| first_indexed | 2024-03-09T03:22:03Z |
| format | Article |
| id | doaj.art-f535f04c5d27472898ea44bcb8a7d0ac |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-03-09T03:22:03Z |
| publishDate | 2021-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-f535f04c5d27472898ea44bcb8a7d0ac2023-12-03T15:09:25ZengMDPI AGBrain Sciences2076-34252021-01-0111216910.3390/brainsci11020169Recognition Memory in Noonan SyndromeFloriana Costanzo0Paolo Alfieri1Cristina Caciolo2Paola Bergonzini3Francesca Perrino4Giuseppe Zampino5Chiara Leoni6Deny Menghini7Maria Cristina Digilio8Marco Tartaglia9Stefano Vicari10Giovanni Augusto Carlesimo11Child and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyChild and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyChild and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyChild and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyCenter for Rare Diseases and Birth Defects, Department of Woman and Child Health, Institute of Pediatrics, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Catholic University of the Sacred Heart, 00168 Rome, ItalyCenter for Rare Diseases and Birth Defects, Department of Woman and Child Health, Institute of Pediatrics, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Catholic University of the Sacred Heart, 00168 Rome, ItalyCenter for Rare Diseases and Birth Defects, Department of Woman and Child Health, Institute of Pediatrics, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Catholic University of the Sacred Heart, 00168 Rome, ItalyChild and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyGenetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyGenetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyChild and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyLaboratory of Clinical and Behavioral Neurology, Santa Lucia Foundation, 00179 Rome, ItalyNoonan syndrome (NS) and the clinically related NS with multiple lentiginous (NMLS) are genetic conditions characterized by upregulated RAS mitogen activated protein kinase (RAS–MAPK) signaling, which is known to impact hippocampus-dependent memory formation and consolidation. The aim of the present study was to provide a detailed characterization of the recognition memory of children and adolescents with NS/NMLS. We compared 18 children and adolescents affected by NS and NMLS with 22 typically developing (TD) children, matched for chronological age and non-verbal Intelligence Quotient (IQ), in two different experimental paradigms, to assess familiarity and recollection: a Process Dissociation Procedure (PDP) and a Task Dissociation Procedure (TDP). Differences in verbal skills between groups, as well as chronological age, were considered in the analysis. Participants with NS and NSML showed reduced recollection in the PDP and impaired associative recognition in the TDP, compared to controls. These results indicate poor recollection in the recognition memory of participants with NS and NSML, which cannot be explained by intellectual disability or language deficits. These results provide evidence of the role of mutations impacting RAS–MAPK signaling in the disruption of hippocampal memory formation and consolidation.https://www.mdpi.com/2076-3425/11/2/169RAS–MAPKPTPN11episodic memoryhippocampal memory processesdevelopmental disorders |
| spellingShingle | Floriana Costanzo Paolo Alfieri Cristina Caciolo Paola Bergonzini Francesca Perrino Giuseppe Zampino Chiara Leoni Deny Menghini Maria Cristina Digilio Marco Tartaglia Stefano Vicari Giovanni Augusto Carlesimo Recognition Memory in Noonan Syndrome Brain Sciences RAS–MAPK PTPN11 episodic memory hippocampal memory processes developmental disorders |
| title | Recognition Memory in Noonan Syndrome |
| title_full | Recognition Memory in Noonan Syndrome |
| title_fullStr | Recognition Memory in Noonan Syndrome |
| title_full_unstemmed | Recognition Memory in Noonan Syndrome |
| title_short | Recognition Memory in Noonan Syndrome |
| title_sort | recognition memory in noonan syndrome |
| topic | RAS–MAPK PTPN11 episodic memory hippocampal memory processes developmental disorders |
| url | https://www.mdpi.com/2076-3425/11/2/169 |
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