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Related Experiment Video

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Novel Object Recognition Test for the Investigation of Learning and Memory in Mice
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Recognition Memory in Noonan Syndrome.

Floriana Costanzo1, Paolo Alfieri1, Cristina Caciolo1

  • 1Child and Adolescent Psychiatric Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Brain Sciences
|February 12, 2021
PubMed
Summary

Children with Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NMLS) exhibit impaired recollection, a key aspect of memory. This deficit in recognition memory is linked to genetic mutations affecting RAS-MAPK signaling pathways.

Keywords:
PTPN11RAS–MAPKdevelopmental disordersepisodic memoryhippocampal memory processes

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Area of Science:

  • Neuroscience
  • Genetics
  • Developmental Biology

Background:

  • Noonan syndrome (NS) and Noonan syndrome with multiple lentigines (NMLS) are genetic disorders.
  • These conditions involve dysregulated RAS mitogen activated protein kinase (RAS-MAPK) signaling.
  • RAS-MAPK signaling impacts hippocampus-dependent memory.

Purpose of the Study:

  • To characterize recognition memory in children and adolescents with NS/NMLS.
  • To investigate the specific components of recognition memory affected, namely familiarity and recollection.
  • To explore the relationship between RAS-MAPK signaling and memory deficits.

Main Methods:

  • Compared 18 children/adolescents with NS/NMLS to 22 typically developing (TD) controls.
  • Used Process Dissociation Procedure (PDP) and Task Dissociation Procedure (TDP) to assess memory.
  • Matched participants on chronological age and non-verbal Intelligence Quotient (IQ), considering verbal skills.

Main Results:

  • Participants with NS/NMLS showed reduced recollection in the PDP.
  • Impaired associative recognition was observed in the TDP for NS/NMLS participants.
  • Deficits were not attributable to intellectual disability or language impairments.

Conclusions:

  • Individuals with NS/NMLS exhibit significant deficits in recollection.
  • RAS-MAPK pathway mutations disrupt hippocampal memory formation and consolidation.
  • These findings highlight the role of specific genetic pathways in cognitive development.