Heterozygous CDKL5 Knockout Female Mice Are a Valuable Animal Model for CDKL5 Disorder

Figure 1

Intermediate Cdkl5 expression levels in the brain of Cdkl5 +/− female mice. (a, b) Western blot analysis of Cdkl5 levels normalized to GAPDH (glyceraldehyde 3-phosphate dehydrogenase) levels in the cortex (Cx), hippocampus (Hp), and cerebellum (Cb) of wild-type (Cdkl5 +/+ ), heterozygous (Cdkl5 +/− ), and homozygous (Cdkl5 −/− ) Cdkl5 KO female mice aged 3–4 months. Immunoblots in (a) are examples from one animal of each experimental group, and the graph shows the relative amounts of Cdkl5 expression in the different brain structures of Cdkl5 +/+ mice. Regardless of the relative amounts, Cdkl5 expression levels were reduced to about 35–46% in Cdkl5 +/− mice in comparison to wild-type mice and absent in Cdkl5 −/− mice in all analyzed brain regions (b). Data in (b) are expressed as a percentage of the values of Cdkl5 +/+ mice. Values are represented as . and (Mann–Whitney test after unpaired -test).

(a)

(b)