Mouse models for down syndrome-associated developmental cognitive disabilities

Chunhong Liu, Pavel V. Belichenko, Li Zhang, Dawei Fu, Alexander M. Kleschevnikov, Antonio Baldini, Stylianos E. Antonarakis, William C. Mobley, Y. Eugene Yu

Research output: Contribution to journalReview articlepeer-review

Abstract

Down syndrome (DS) is mainly caused by the presence of an extra copy of human chromosome 21 (Hsa21) and is a leading genetic cause for developmental cognitive disabilities in humans. The mouse is a premier model organism for DS because the regions on Hsa21 are syntenically conserved with three regions in the mouse genome, which are located on mouse chromosome 10 (Mmu10), Mmu16 and Mmu17. With the advance of chromosomal manipulation technologies, new mouse mutants have been generated to mimic DS at both the genotypic and phenotypic levels. Further mouse-based molecular genetic studies in the future may lead to the unraveling of the mechanisms underlying DS-associated developmental cognitive disabilities, which would lay the groundwork for developing effective treatments for this phenotypic manifestation. In this review, we will discuss recent progress and future challenges in modeling DS-associated developmental cognitive disability in mice with an emphasis on hippocampus-related phenotypes.

Original languageEnglish (US)
Pages (from-to)404-413
Number of pages10
JournalDevelopmental Neuroscience
Volume33
Issue number5
DOIs
StatePublished - Nov 2011

Keywords

  • Developmental cognitive disabilities
  • Down syndrome
  • Human trisomy 21
  • Mouse models
  • Targeted chromosome manipulation

ASJC Scopus subject areas

  • Neurology
  • Developmental Neuroscience

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