The generation of a conditional Fmr1 knock out mouse model to study Fmrp function in vivo.
Neurobiol Dis
; 21(3): 549-55, 2006 Mar.
Article
en En
| MEDLINE
| ID: mdl-16257225
ABSTRACT
The FMR1 gene, mutated in Fragile X syndrome patients, has been modeled in mice with a neomycin cassette inserted in exon 5 of the mouse Fmr1 gene creating an Fmr1 knockout (Fmr1 KO) allele. This results in animals lacking Fmr1 protein (Fmrp) expression in all tissues. We have created a new, more versatile Fmr1 in vivo KO model (Fmr1 KO2) and generated conditional Fmr1 KO (CKO) mice by flanking the promoter and first exon of Fmr1 with lox P sites. This enables us to create a null allele in specific cell types and at specific time points by crossing Fmr1 CKO mice with tissue specific or inducible cre-recombinase expressing mice. The new Fmr1 KO2 line does not express any Fmrp and also lacks detectable Fmr1 transcripts. Crossing the Fmr1 CKO line with a Purkinje cell-specific cre-recombinase expresser produces mice that are null for Fmr1 in Purkinje neurons but wild type in all other cell types.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Células de Purkinje
/
Modelos Animales de Enfermedad
/
Proteína de la Discapacidad Intelectual del Síndrome del Cromosoma X Frágil
Límite:
Animals
Idioma:
En
Revista:
Neurobiol Dis
Asunto de la revista:
NEUROLOGIA
Año:
2006
Tipo del documento:
Article
País de afiliación:
Países Bajos