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. 2015 Jan 26;290(12):7871–7886. doi: 10.1074/jbc.M114.605386

FIGURE 1.

FIGURE 1.

Targeted disruption of Abcb6 results in a pleiotropic phenotype. a, schematic presentation of the wild-type locus (Abcb6 genomic fragment), targeted construct (targeting construct), and deleted locus (disrupted allele). Boxes represent exons. Arrows represent genotyping primer priming site. F, forward; R, reverse primers. b, PCR analysis of genomic DNA from tails. c, expression of Abcb6 mRNA in liver as determined by real time RT-PCR. Mean ± S.E. data from three independent measurements are shown. *, significantly different from Abcb6+/+ mice; p < 0.001. #, significantly different from Abcb6+/− mice; p < 0.001. d, immunostaining for Abcb6 in liver. Top panel Abcb6 immunostaining; bottom panel actin immunostaining (loading control). e, HPLC analysis of hepatic heme levels. Mean ± S.E. data from three independent measurements are shown. Heme concentration was calculated from a heme standard curve. f, expression of hepatic enzymes involved in heme synthesis as determined by real time RT-PCR in Abcb6+/+ (WT) and Abcb6−/− (KO) mouse. Mean ± S.E. data from three independent measurements are shown. g, Mendelian inheritance pattern of Abcb6 allele. h, representative photo of age-matched (6 weeks) and sex-matched (males) Abcb6−/− short stature mice and mice with no gross abnormal phenotype presented alongside a ruler for reference. ALAS1, aminolevulinic acid synthase; ALAD, aminolevulinate dehydratase; UROS, uroporphyrinogen III synthase; UROD, uroporphyrinogen III decarboxylase; CPOX, coproporphyrinogen oxidase; FECH, ferrochelatase.