The Cytoplasm: Peroxisomes and Peroxisomal Diseases

Peroxisomes are ubiquitous organelles that contain catalase and oxidative enzymes producing H2O2. Depending on cell type, their number, shape, and size vary. By electron microscopy, peroxisomes have a single membrane that encloses a dense matrix that cont

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mary tubules (5 nm inner diameter) are arranged in a circle and form a centrally located secondary tubule (20 nm outer diameter). Remarkably, both urate and xanthine oxidase were restricted to the lumen of primary tubules. An extreme example of compartmentation was observed for angular peroxisomes of beef kidney. The crystalline core contained urate oxidase, the noncrystalline central region of the matrix D-amino acid oxidase, the peripheral matrix region catalase and α-hydroxy acid oxidase A, and the marginal dense plates close but separated from the peroxisomal membrane α-hydroxy acid oxidase B. Panel D shows the presence of the 70 kDa peroxisomal membrane protein (PMP-70). Under conditions of peroxisome proliferation, the PMP-70 was found not only along the peroxisomal membrane (panel D) but additionally in membranous loops in continuity with it.

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Magnification: ×50,000 (A); ×63,000 (B–D) M. Pavelka, J. Roth, Functional Ultrastructure: Atlas of Tissue Biology and Pathology, Third Edition, DOI 10.1007/978-3-7091-1830-6_8, © Springer-Verlag Vienna 2015

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The Cytoplasm: Peroxisomes and Peroxisomal Diseases

PEROXISOME BIOGENESIS All peroxisomal membrane and matrix proteins are encoded by the nucleus and synthesized on polysomes. Peroxisome biogenesis is multifaceted, and two models prevail that are not mutually exclusive: de novo biogenesis involvin