While transcriptional changes in lipid metabolism genes have not been confirmed in DD patients and the lipid content and composition of the kidney cortex in ClC-5 KO mice or DD patients have yet to be quantified, we have observed changes in the cellular distribution of the cholesterol marker filipin in PTs isolated from ClC-5 KO male mice and heterozygous female mice when compared with WT controls (unpublished data). This evidence concerns the gene CLCN5 and dentin dysplasia.