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The generation of a conditional Fmr1 knock out mouse model to study Fmrp function in vivo.

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.

Pubmed ID: 16257225


  • Mientjes EJ
  • Nieuwenhuizen I
  • Kirkpatrick L
  • Zu T
  • Hoogeveen-Westerveld M
  • Severijnen L
  • RifĂ© M
  • Willemsen R
  • Nelson DL
  • Oostra BA


Neurobiology of disease

Publication Data

March 28, 2006

Associated Grants

  • Agency: NICHD NIH HHS, Id: HD024064
  • Agency: NICHD NIH HHS, Id: HD29256
  • Agency: NICHD NIH HHS, Id: R01 HD38038

Mesh Terms

  • Animals
  • Blotting, Western
  • Disease Models, Animal
  • Fragile X Mental Retardation Protein
  • Fragile X Syndrome
  • Immunohistochemistry
  • Mice
  • Mice, Knockout
  • Purkinje Cells
  • Reverse Transcriptase Polymerase Chain Reaction