TY - JOUR
T1 - Developmental abnormalities and age-related neurodegeneration in a mouse model of Down syndrome
AU - Holtzman, David M.
AU - Santucci, Daniela
AU - Kilbridge, Joshua
AU - Chua-Couzens, Jane
AU - Fontana, David J.
AU - Daniels, Scott E.
AU - Johnson, Randolph M.
AU - Chen, Karen
AU - Sun, Yuling
AU - Carlson, Elaine
AU - Alleva, Enrico
AU - Epstein, Charles J.
AU - Mobley, William C.
PY - 1996/11/12
Y1 - 1996/11/12
N2 - To study the pathogenesis of central nervous system abnormalities in Down syndrome (DS), we have analyzed a new genetic model of DS, the partial trisomy 16 (Ts65Dn) mouse. Ts65Dn mice have an extra copy of the distal aspect of mouse chromosome 16, a segment homologous to human chromosome 21 that contains much of the genetic material responsible for the DS phenotype. Ts65Dn mice show developmental delay during the postnatal period as well as abnormal behaviors in both young and adult animals that may be analogous to mental retardation. Though the Ts65Dn brain is normal on gross examination, there is age-related degeneration of septohippocampal cholinergic neurons and astrocytic hypertrophy, markers of the Alzheimer disease pathology that is present in elderly DS individuals. These findings suggest that Ts65Dn mice may be used to study certain developmental and degenerative abnormalities in the DS brain.
AB - To study the pathogenesis of central nervous system abnormalities in Down syndrome (DS), we have analyzed a new genetic model of DS, the partial trisomy 16 (Ts65Dn) mouse. Ts65Dn mice have an extra copy of the distal aspect of mouse chromosome 16, a segment homologous to human chromosome 21 that contains much of the genetic material responsible for the DS phenotype. Ts65Dn mice show developmental delay during the postnatal period as well as abnormal behaviors in both young and adult animals that may be analogous to mental retardation. Though the Ts65Dn brain is normal on gross examination, there is age-related degeneration of septohippocampal cholinergic neurons and astrocytic hypertrophy, markers of the Alzheimer disease pathology that is present in elderly DS individuals. These findings suggest that Ts65Dn mice may be used to study certain developmental and degenerative abnormalities in the DS brain.
UR - http://www.scopus.com/inward/record.url?scp=10544243361&partnerID=8YFLogxK
U2 - 10.1073/pnas.93.23.13333
DO - 10.1073/pnas.93.23.13333
M3 - Article
C2 - 8917591
AN - SCOPUS:10544243361
SN - 0027-8424
VL - 93
SP - 13333
EP - 13338
JO - Proceedings of the National Academy of Sciences of the United States of America
JF - Proceedings of the National Academy of Sciences of the United States of America
IS - 23
ER -