FIGURE 1.

Expression of PMP34 in murine embryonic and adult life. (A) Northern analysis of RNA isolated from liver of age matched female (F) and male (M) mice of the indicated genotypes. Migration of ribosomal markers indicated at the left. (B–E) PMP34 expression in Slc25a17^+/– embryos at different prenatal stages or in adult mice as revealed by promoter-driven β-galactosidase reporter expression: whole mount staining at E₇.₅ (B) and E₁₂.₅ (C); stained cryosections (10 μm) counterstained with Nuclear Fast Red from liver (D) and kidney (E) (Scale bar 10 μm; stainings of WT samples were negative). (F) β-galactosidase activity in tissues derived from 1 month old ^+/– male (black bars) or adult ^–/male (gray bars). Values are represented as mean ± SD of separate measurements on two mice/genotype, except for liver (n = 5 for ^–/–, n = 3 for ^+/–). Galactosidase activities in tissues of WT mice were less then 0.05 mU/mg protein (not shown). Hard. Gl., Harderian gland; prep. Gl., preputial gland; CBR, cerebrum; CBL, cerebellum; duodenum, duodenal mucosa; BAT, brown adipose tissue. Panel G, PMP34 deficiency at the protein level. Total liver homogenates of adult wild type (WT) or PMP34-deficient (KO) mice, all containing equal amounts of protein, were processed for SDS-PAGE, Western blotting, and sequential immunoblot analysis with antisera specific for PMP34 and β-actin. The migration points of relevant molecular mass markers (expressed in kDa) are shown on the left. The arrows mark specific immunoreactive bands.