Peroxisome Morphology in Pathologies
Peroxisomes are ubiquitous and heterogeneous multi-purpose organelles, which are indispensable for human health and development. The invention of specific cytochemical staining methods for peroxisomes revealed their high plasticity and ability to alter th
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Peroxisome Morphology in Pathologies Michael Schrader, Ineˆs Castro, H. Dariush Fahimi, and Markus Islinger
Abstract
Peroxisomes are ubiquitous and heterogeneous multi-purpose organelles, which are indispensable for human health and development. The invention of specific cytochemical staining methods for peroxisomes revealed their high plasticity and ability to alter their morphology in response to environmental cues. Peroxisome dynamics depend on peroxisomal morphology proteins such as Pex11p, DLP1/Drp1, Fis1, Mff, and GDAP1 which are partially shared with mitochondria. Here, we address variations of peroxisome morphology in the healthy organism and summarize findings on altered organelle morphology in peroxisomal disorders. We highlight recent insights in novel disorders with defects in peroxisome morphology proteins and alterations of peroxisomes during stress and signaling, as well as secondary alterations in liver disease and cancer.
M. Schrader (*) College of Life and Environmental Sciences, Biosciences, University of Exeter, Geoffrey Pope Building, Stocker Road, Exeter EX4 4QD, UK Department of Biology, Centre for Cell Biology, University of Aveiro, Campus Universita´rio de Santiago, 3810-193 Aveiro, Portugal e-mail: [email protected] I. Castro College of Life and Environmental Sciences, Biosciences, University of Exeter, Geoffrey Pope Building, Stocker Road, Exeter EX4 4QD, UK H.D. Fahimi Division of Medical Cell Biology, Department of Anatomy and Cell Biology, University of Heidelberg, 69120 Heidelberg, Germany M. Islinger Department of Neuroanatomy, Center for Biomedicine and Medical Technology Mannheim, University of Heidelberg, 68167 Mannheim, Germany C. Brocard and A. Hartig (eds.), Molecular Machines Involved in Peroxisome Biogenesis and Maintenance, DOI 10.1007/978-3-7091-1788-0_7, # Springer-Verlag Wien 2014
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Keywords
Peroxisomes • Organelle dynamics • Biogenesis disorders • Dynamin • Pex11 • Mff • GDAP1
Abbreviations AOX CMT D-BP DAB DHA DLP1/Drp1 ER GDAP1 Mff PBD PEX PMP PPAR PTS ROS SED
7.1
Acyl-CoA oxidase Charcot-Marie-Tooth disease D-bifunctional protein Diaminobenzidine Docosahexaenoic acid Dynamin-like/related protein 1 Endoplasmic reticulum Ganglioside-induced differentiation associated protein 1 Mitochondrial fission factor Peroxisome biogenesis disorder Peroxin Peroxisomal membrane protein Peroxisome proliferator activated receptor Peroxisomal targeting signal Reactive oxygen species Single enzyme deficiency
Variation of Peroxisome Morphology in the Healthy Organism
Peroxisomes (microbodies) were first described morphologically in 1954 by the Swedish PhD student and later electron microscopy pioneer Johannes Rhodin who performed ultrastructural studies of the mouse kidney (Rhodin 1954). They belong to the basic equipment of the eukaryotic cell and are found in mammals, plants, and fungi. In ultrastructure studies, they present themselves as single-membrane bound subcellular compartments with a fine granular matrix harboring diverse enzymes and
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