1. Rett Syndrome Neurobiology of Disease

2. Rett Syndrome 2nd most common genetic cause of Mental Retardation Early development normal  loss of language, cognitive and motor abilities Single gene defect: Mecp2 KO (Jaenisch, Bird) recapitulates disease, as does forebrain neuron selective KO.

3. http://www.ncbi.nlm.nih.gov/disease/chr21-Y.html

4. Zohgbi, 2003

5. MeCP2 represses gene expression

6. Cre/lox system http://www.bioteach.ubc.ca/MolecularBiology/TargetingYourDNAWithTheCreloxSystem /

7. But remarkably little effect on gene expression detected in microarray study of mouse KO Tudor et al. 2002 MeCP2 represses gene expression

8. Shabazian et al. 2002

9. Luikenhuis, Giacometti, Beard, Jaenisch (2004) Fig 1.

10. Luikenhuis, Giacometti, Beard, Jaenisch (2004) Fig 2.

11. Luikenhuis, Giacometti, Beard, Jaenisch (2004) Fig 3.

12. Luikenhuis, Giacometti, Beard, Jaenisch (2004)Fig 4.

13. Dani et al. (2005)Fig 1.

14. Dani et al. (2005)Fig 2.

15. Dani et al. (2005) Fig 3.

16. Dani et al. (2005) Fig 4.

17. Dani et al. (2005) Fig 5.