Review Article

Rett Syndrome: Coming to Terms with Treatment

Table 5

Rett syndrome animal models of Rett syndrome.

Mouse model typeGenetic compositionReference

Null mutation
Tm1.1BirdExon 3-4 deletion[139]
Tml1.1JaeExon 3 deletion[140]
Tm1.PpltMethyl-binding domain deletion[141]
2loxB TH-Cre Dopaminergic/noradrenergic neurons[142]
2loxB PET1-Cre Serotonergic neurons[143]
2loxJ hGFAP-CreT2 Astrocytes[144]

Truncated mutation
Tm1HzoNucleotide 308 truncation[145]

Human point mutation
Tm1.1CoyleR168X[146]
Tm1.1VnarA140V[147]
Tm1.1HupR168X[148]
Tm1.1JoezT158A[149]
Tm1.1IRSFR255XUnpublished