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No entry without protein recycling: RUB researchers discover new coherence in enzyme transport
February 13, 2012
Journal of Biological Chemistry: Linking of import and export The group of Prof. Dr. Ralf Erdmann at the Ruhr-Universität Bochum (Faculty of Medicine, Department of Systems Biochemistry) discovered a connection of peroxisomal protein import and receptor export. In the Journal of Biological Chemistry, they disclosed that enzymes only get imported into certain cell organelles (peroxisomes) upon coupling of their import to the recycling of their transport protein (receptor). Multi-functional tool peroxisome Peroxisomes do not have their own DNA. Thus, all peroxisomal proteins are coded within the nucleus and imported into the peroxisome after their synthesis is completed. The Erdmann lab investigates this process in detail. Peroxisomes contain more than 50 various enzymes in total which e.g. decompose fatty acids and dispose hydrogen peroxide or plasmalogens, the main phospholipid of the white matter of the brain. A disruption of their function does not only cause severe metabolic disorders, it can even lead to death of newborns. Export signal controls recycling Dynamic receptors recognize and escort the enzymes destined for the peroxisome to the organelle where they attach to the membrane. Then the receptor-enzyme complex disassembles and the enzyme is transported across the peroxisomal membrane. Afterwards, the receptor is transported from the membrane back to the cytosol. This recycling is controlled by the attachment of the small protein ubiquitin to the receptor, which functions as an export signal. Modification as a safety barrier The team of Prof. Dr. Ralf Erdmann studied a certain peroxisomal receptor, which consists of a targeting unit (Pex18p) and an enzyme-binding unit (Pex7p). The scientists discovered that ubiquitin modifies Pex18p in order to enable the receptor to return to the intracellular liquid. Only when the targeting unit of the receptor is exported from the membrane, the import of the cargo-loaded enzyme-binding unit takes place. This result supports the export-driven-import model, previously proposed by the Erdmann group. Ruhr-University Bochum

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Peroxisomal Disorders and Regulation of Genes (Advances in Experimental Medicine and Biology)
by Frank Roels (Editor), Myriam Baes (Editor), Sylvia De Bie (Editor)
In most peroxisomal disorders the nervous system is severely affected which explains the clinical and community burden they represent. This is the first book to focus not only on the mutations causing these inherited illnesses, but also on mechanisms that regulate, suppress or enhance expression of genes and their products (enzymes). Indeed since the success and completion of the Human Genome Project all genes (coding DNA sequences) are known. However, of many, their function, and the role of the gene product has not been determined. An example is X-linked adrenoleukodystrophy, the most frequent peroxisomal disorder. Children are born healthy, but in more than 1 out of 3, demyelination of the brain starts unpredictably and they die in a vegetative state. The gene mutated in most families...
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Adrenoleukodystrophy and Other Peroxisomal Disorders: Clinical, Biomedical, Genetic and Therapeutic Aspects - International Workshop Proceedings (International Congress)
by Graziella Uziel (Editor), etc. (Editor), Ronald J.A. Wanders (Editor), Marco Cappa (Editor)
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Gale Encyclopedia of Medicine: Peroxisomal disorders
by Judith Sims (Author)
The article is excerpted from Gale Encyclopedia of Medicine. Consult the second edition of this authoritative, comprehensive, in-depth medical guide for information on more than 1,700 medical topics in language accessible to adult laypersons. Presented in a single alphabetical sequence, articles range in length from one or two paragraphs for minor topics, to several pages or more for major topics. Disease/disorder articles typically cover definition; description; causes and symptoms; diagnosis; treatments; prevention; and more. Test/treatment articles typically cover definition; purposes; precautions; preparation; risks; normal and abnormal results; and much more. This second edition includes more than 200 new entries, 300 updated entries, approximately 650 color images...
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Peroxisomal disorders: An entry from Thomson Gale's Gale Encyclopedia of Children's Health: Infancy through Adolescence
by Judith Sims (Author), Stephanie Sherk (Author)
Avoiding hard-to-understand medical jargon, the four-volume “Gale Encyclopedia of Children’s Health” uses language that parents can understand, while still providing enough depth to benefit today's health science students. The set provides in-depth coverage of pediatric diseases and disorders, along with issues related to physical and cognitive/behavioral development.
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![Seasonal variability of oxidative biomarkers, lysosomal parameters, metallothioneins and peroxisomal enzymes in the Mediterranean mussel Mytilus ... Adriatic Sea [An article from: Chemosphere]](http://ecx.images-amazon.com/images/I/51M6G4MFGFL._SX120__PC__PE00_.jpg)
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Seasonal variability of oxidative biomarkers, lysosomal parameters, metallothioneins and peroxisomal enzymes in the Mediterranean mussel Mytilus ... Adriatic Sea [An article from: Chemosphere]
by R. Bocchetti (Author), F. Regoli (Author)
This digital document is a journal article from Chemosphere, published by Elsevier in 2006. The article is delivered in HTML format and is available in your Amazon.com Media Library immediately after purchase. You can view it with any web browser.
Description: The Mediterranean mussel, Mytilus galloprovincialis, is a classical sentinel organism for monitoring the biological effects of contaminants through the use of molecular and cellular biomarkers. These biological responses can be modulated also by seasonal changes of both environmental and biological factors, potentially influencing responsiveness and sensitivity to pollutants. The aim of this study was to characterize in a reference mussel population from the Adriatic Sea, the natural fluctuations of several oxidative...
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Peroxisomal Disorders: An entry from Gale's Gale Encyclopedia of Medicine, 3rd ed.
by Judith Sims (Author), Teresa Odle (Author)
This digital document is an article from Gale Encyclopedia of Medicine, 3rd ed., brought to you by Gale®, a part of Cengage Learning, a world leader in e-research and educational publishing for libraries, schools and businesses. The length of the article is 1760 words. The article is delivered in HTML format and is available in your Amazon.com Digital Locker immediately after purchase. You can view it with any web browser. The third edition of this authoritative, comprehensive, in-depth medical guide features information on medical topics in language accessible to adult laypersons. Disease/disorder articles typically cover definition; description; causes and symptoms; diagnosis; treatments; prevention; and more. Test/treatment articles typically cover definition; purposes;...
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Peroxisomal: Webster's Timeline History, 1968 - 2007
by Icon Group International (Author)
Webster's bibliographic and event-based timelines are comprehensive in scope, covering virtually all topics, geographic locations and people. They do so from a linguistic point of view, and in the case of this book, the focus is on "Peroxisomal," including when used in literature (e.g. all authors that might have Peroxisomal in their name). As such, this book represents the largest compilation of timeline events associated with Peroxisomal when it is used in proper noun form. Webster's timelines cover bibliographic citations, patented inventions, as well as non-conventional and alternative meanings which capture ambiguities in usage. These furthermore cover all parts of speech (possessive, institutional usage, geographic usage) and contexts, including pop culture, the arts, social...
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Diagnosis of Human Peroxisomal Disorders: A Handbook - Journal of Inherited Metabolic Disease 18:1, 1995
by F. Roels (Author), S. De Bie (Author), Frank Roels (Editor), Sylvia De Bie (Editor), R.B.H. Schutgens (Editor), G.T.N. Besley (Editor)
Peroxisomal disorders constitute a major research front in clinical genetics, paediatrics and cell biology. Since 1983, the metabolic defect in some 20 different peroxisomal disorders has been described. The best known conditions include Zellweger syndrome, rhizomelic chondrodysplasia punctata and X-linked adrenoleukodystrophy and, in the most recent edition of The Metabolic and Molecular Basis Inherited Disease, edited by Scriver and colleagues, more than 100 pages are now devoted to the subject. Progress in our understanding of these conditions, and their diagnosis, results from the application of a variety of laboratory investigations. These include microscopic studies, analysis of metabolites (very long-chain fatty acids, bile acids, and plasmalogens), enzyme studies...
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Biochemical analysis of the regulation of vesicle fusion, peroxisomal protein import and proteolytic processing in eukaryotes.
by Antionette L Williams (Author)
My dissertation research provides major contributions to the current understanding of intracellular protein trafficking in two important areas: vesicular fusion and peroxisome biogenesis. I began with a study of SNAREs, highly conserved proteins that form the core fusion machinery within secretory and endosomal trafficking systems. I examined the regulation of an integral member of this protein machinery, syntaxin 5, by its mammalian endoplasmic reticulum/Golgi Sec1/Munc18 protein binding partner rSly1. To address their functional relationship, I produced a conformation-specific monoclonal antibody to use in immunostaining experiments and in vitro ER-to-Golgi transport assays. Results from the manipulation of rSly1/syntaxin 5 interactions indicate that rSly1 function is intimately...
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Mechanism of peroxisomal targeting of human soluble epoxide hydrolase (hsEH) and its effect on hsEH stability and activity.
by Beibei (Belinda) Luo (Author)
Soluble epoxide hydrolase (sEH) is a homodimeric enzyme with a C-terminal epoxide hydrolase domain and an N-terminal phosphatase domain. sEH has been suggested to play a role in a variety of biological processes, like inflammation, hypertension, cell signaling and cholesterol synthesis, by modulating levels of its endogenous substrates. However, its biological significance in human remains unclear. Part of this unknown is due to controversy regarding human soluble epoxide hydrolase (hsEH) subcellular localization. Despite the putative peroxisomal targeting sequences on the protein sequence, hsEH is dual localized in both peroxisomes and cytosol in human tissues. The unusual dual localization of hsEH, together with the differential localization of the substrates of hsEH C-terminal domain...
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