By Cecile Brocard, Andreas Hartig

ISBN-10: 3709117879

ISBN-13: 9783709117873

ISBN-10: 3709117887

ISBN-13: 9783709117880

In eukaryotes, lipid metabolism calls for the functionality of peroxisomes. those multitasking organelles also are a part of species-specific pathways similar to the glyoxylate cycle in yeast and crops or the synthesis of ether lipid in mammals. Proteins required for the biogenesis of peroxisomes usually gather in huge molecular complexes, which perform membrane formation, protein shipping, peroxisome duplication and - inheritance in the course of mobilephone department. Peroxisomal functionality is key for all times. Mutations in PEX genes, encoding for biogenesis components, are frequently linked to deadly issues. The organization of peroxisomes with different organelles indicates an in depth participation in organellar crosstalk. This ebook represents a cutting-edge evaluation within the box of peroxisome examine encompassing the mobilephone and molecular biology of peroxisome biogenesis and its illnesses, the protein complexes serious about this technique and the trendy applied sciences utilized to review them. The e-book is meant for graduate scholars, researchers and academics in biochemistry, molecular and mobilephone biology with a biomedical background.

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Extra info for Molecular Machines Involved in Peroxisome Biogenesis and Maintenance

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Furthermore, Pex11αÀ/À hepatocytes store more lipids than control mice (shown in f, g after a 48-h fasting period; reproduced from Weng et al. (2013) with permission from the American Physiological Society). Ct ¼ sections from control mice Cre expression because a similar pathology is observed in mice with a generalized peroxisomal β-oxidation defect (Mfp2À/À mice; Huyghe et al. 2006). This also indicates that the neutral lipid accumulation in peroxisome-deficient cells is a consequence of impaired β-oxidation although the precise origin and nature of the lipids needs to be determined.

In this way prostanoids are inactivated by cleavage of their carboxy-side chains. The general theme of peroxisomal beta-oxidation is that peroxisomes perform one or more cycles of beta-oxidation and release the products to the cytosol for subsequent metabolism. In some cases peroxisomes catalyse a single cycle of betaoxidation. This is true for the bile acid intermediates di- and trihydroxycholestanoic acid which undergo a single cycle of beta-oxidation generating cholic acid and chenodeoxycholic acid in their CoA-ester form.

The latter however display deregulated expression of several genes at the mRNA and protein level. The cellular changes in Pex11β+/À mice did not markedly affect CNS functioning because the mice are macroscopically indistinguishable from wild types and they are fertile. This is in sharp contrast with the phenotype of mice with peroxisome import defects in neural cells that show reduced growth, fertility, coordination and motor defects as described above (Hulshagen et al. 2008; Muller et al. 2011).

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Molecular Machines Involved in Peroxisome Biogenesis and Maintenance by Cecile Brocard, Andreas Hartig

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