Transgenic mouse model for the fragile X syndrome.
Transgenic fragile X knockout mice have been constructed to provide an animal model to study the physiologic function of the fragile X gene (FMR1) and to gain more insight into the clinical phenotype caused by the absence of the fragile X protein. Initial experiments suggested that the knockout mice show macroorchidism and cognitive and behavioral deficits, abnormalities comparable to those of human fragile X patients. In the present study, we have extended our experiments, and conclude that the Fmr1 knockout mouse is a reliable transgenic model to study the fragile X syndrome.
Kooy
R F
RF
Department of Medical Genetics, University of Antwerp, Belgium.
D'Hooge
R
R
Reyniers
E
E
Bakker
C E
CE
Nagels
G
G
De Boulle
K
K
Storm
K
K
Clincke
G
G
De Deyn
P P
PP
Oostra
B A
BA
Willems
P J
PJ
D000375:Aging / Q000502:physiology; D000818:Animals; D051860:Fragile X Messenger Ribonucleoprotein 1; D005600:Fragile X Syndrome / Q000235:genetics* / Q000503:physiopathology* / Q000523:psychology; D006801:Humans; D008297:Male; D018782:Maze Learning; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000502:physiology; D009929:Organ Size; D016601:RNA-Binding Proteins; D012016:Reference Values; D013737:Testis / Q000254:growth & development
D051860:Fragile X Messenger Ribonucleoprotein 1; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins; C497683:FMR1 protein, human; C497684:Fmr1 protein, mouse