Figure 2.

Expression of FlexCln3 allele in all cells reduces storage accumulation in Cln3^{Δex78/Δex78} brain

(A) In the presence of Cre, the FlexCln3 allele undergoes inversion (step 1) and excision (step 2) and is expressed under control of its endogenous promoter. (B) PCR of genomic DNA from the hippocampus using orientation-specific primers confirms that the FlexCln3 is in the forward orientation only in the presence of Cre (see Figure S7 for uncut gels). (C) In the presence of Zp3-Cre, which drives ubiquitous recombination, the FlexCln3 allele results in expression levels that are 40.0% ± 0.4% of heterozygous controls. (D) Subunit C of the mitochondrial ATP synthase (SCMAS) accumulates in the hippocampus of Cln3^{Δex78/Δex78} and FlexCln3/Cln3^{Δex78/Δex78} mice. Storage is reduced in mice with the FlexCln3 allele in the forward confirmation.