Total RNA (20 μg) froml-PBE−/− and wild type littermate mice on control (−) or ciprofibrate (Cipro, 0.0125% w/w)-containing diet (+) for 2 weeks was hybridized with32P-labeled cDNA probes as indicated. First, it may be required for the inductive signal that induces the outgrowth of the ureter from the mesonephros. These observations implied that AOX is responsible for the metabolic degradation of PPARα ligands. In this study, the function of enoyl-CoA hydratase/l-3-hydroxyacyl-CoA dehydrogenase (l-PBE), the second enzyme of this peroxisomal β-oxidation system, was investigated by disrupting its gene. They are characterized by the presence of the paired domain, a conserved amino acid motif with DNA-binding activity. Consistent with the expression of WT1 in the inner portion of the WT1+/+ retina, a significant fraction of cells was lost apoptosis in the developing retinal ganglion cells of mutant E18 embryos. Aniridia. Aniridia is a severe eye disease characterized by iris hypoplasia.
First, these algorithms should have data-parallel computations and repeated operations that are amenable to hardware implementation. Analysis of liver samples by SDS-polyacrylamide gel electrophoresis showed no increase in the 78-kilodalton l-PBE protein in l-PBE−/− mice treated with a peroxisome proliferator (Fig.8). View larger version: Figure 7 Response of PBE−/− and wild type littermate mice to a peroxisome proliferator. 1. Neufeld EF, Muenzer J. The mucopolysaccharidoses. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. Nature. 2006;441:880–884. [PubMed]40. Hara T, Nakamura K, Matsui M, Yamamoto A, Nakahara Y, Suzuki-Migishima R, Yokoyama M, Mishima K, Saito I, Okano H, Mizushima N. Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. The spontaneous increase in l-PBE mRNA in AOX−/− mouse liver is comparable in magnitude to that occurring in ciprofibrate-treated wild type mouse (Fig. 6). We also found ∼5-fold increase in d-PBE mRNA level in the liver of both wild type and l-PBE−/− mice treated with ciprofibrate.
Both sporadic and familial cases with autosomal dominant inheritance have been reported. Both male and female homozygous mice were fertile. Exons 2–5 (E2 to E5) in the l-PBE gene are shown as closed boxes. Mutations in four out nine characterized Pax genes have been associated with either congenital human diseases such as Waardenburg syndrome (Pax3), Aniridia (Pax6), Peter’s anomaly (Pax6), renal coloboma syndrome (Pax2), or spontaneous mouse mutants, which all show defects in development. Screening of 100 G418 ganciclovir-resistant colonies by Southern blotting yielded 5 homologous recombinants for a targeting frequency of 5%. ES cells from two different colonies were injected into C57BL/6J blastocysts to generate chimeric animals. These anomalies include vesico-ureteral reflux (VUR), auditory anomalies, CNS anomalies, and skin and joint anomalies (8). Patients with mutations in Pax2 have colobomatous eye defects.