%0 Journal Article %J Neurobiol Dis %D 2006 %T The generation of a conditional Fmr1 knock out mouse model to study Fmrp function in vivo. %A Mientjes, E J %A Nieuwenhuizen, I %A Kirkpatrick, L %A Zu, T %A Hoogeveen-Westerveld, M %A Severijnen, L %A Rifé, M %A Willemsen, R %A Nelson, D L %A Oostra, B A %K Animals %K Blotting, Western %K Disease Models, Animal %K Fragile X Mental Retardation Protein %K Fragile X Syndrome %K Immunohistochemistry %K Mice %K Mice, Knockout %K Purkinje Cells %K Reverse Transcriptase Polymerase Chain Reaction %X

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.

%B Neurobiol Dis %V 21 %P 549-55 %8 2006 Mar %G eng %N 3 %1 https://www.ncbi.nlm.nih.gov/pubmed/16257225?dopt=Abstract %R 10.1016/j.nbd.2005.08.019