TY - JOUR
T1 - Mouse models for down syndrome-associated developmental cognitive disabilities
AU - Liu, Chunhong
AU - Belichenko, Pavel V.
AU - Zhang, Li
AU - Fu, Dawei
AU - Kleschevnikov, Alexander M.
AU - Baldini, Antonio
AU - Antonarakis, Stylianos E.
AU - Mobley, William C.
AU - Yu, Y. Eugene
PY - 2011/11
Y1 - 2011/11
N2 - 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.
AB - 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.
KW - Developmental cognitive disabilities
KW - Down syndrome
KW - Human trisomy 21
KW - Mouse models
KW - Targeted chromosome manipulation
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U2 - 10.1159/000329422
DO - 10.1159/000329422
M3 - Article
C2 - 21865664
AN - SCOPUS:83055172911
VL - 33
SP - 404
EP - 413
JO - Developmental Neuroscience
JF - Developmental Neuroscience
SN - 0378-5866
IS - 5
ER -