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Chapter 37 - Peroxisomal diseases
- from Section IV - Metabolic liver disease
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- By Paul A. Watkins, Johns Hopkins University School of Medicine, Kennedy Krieger Institute, Baltimore, MD, USA, Kathleen B. Schwarz, Johns Hopkins University School of Medicine; Pediatric Liver Center, Johns Hopkins Hospital, Baltimore, MD, USA
- Edited by Frederick J. Suchy, University of Colorado Medical Center, Ronald J. Sokol, University of Colorado Medical Center, William F. Balistreri
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- Book:
- Liver Disease in Children
- Published online:
- 05 March 2014
- Print publication:
- 20 February 2014, pp 649-663
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Summary
General aspects of peroxisomes
Peroxisomes were first identified in renal proximal tubule cells by a Swedish graduate student in 1954. Initially called microbodies, these organelles were studied intensively by de Duve and coworkers. Because they contained enzymes that both produced (e.g. amino acid and urate oxidases) and degraded (e.g. catalase) hydrogen peroxide, de Duve proposed the name peroxisomes [1]. Microbodies found in some lower organisms and plants were named for the specialized functions that they carry out. For example, glyoxysomes of fungi and plants contain the five enzymes of the glyoxylate cycle and glycosomes house the enzymes of glycolysis in trypanosomes. While peroxisomes have been found in essentially all plant and animal cells with the exception of mature erythrocytes, they range in size from about 0.1μm (microperoxisomes of intestine and brain) up to 1.0μm (characteristic of hepatic and renal peroxisomes; range: 0.2–1.0μm) (Figure 37.1).
A single lipid bilayer comprises the peroxisomal membrane. The organelle’s matrix is finely granular, but microcrystalline cores of urate oxidase are present in the hepatic peroxisomes of some species (e.g. rats). No cores are found in human peroxisomes as humans lack urate oxidase. Unlike chloroplasts and mitochondria, peroxisomes contain no DNA although it has been speculated that all three organelles evolved from endosymbionts. Since the discovery of peroxisomes, numerous membrane proteins and matrix enzymes have been identified. Much research on peroxisomes has been fueled by the identification of patients whose cells lack either normal appearing organelles or one or more peroxisomal metabolic functions.
35 - Peroxisomal Diseases
- from SECTION IV - METABOLIC LIVER DISEASE
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- By Paul A. Watkins, M.D., Ph.D., Professor, Department of Neurology, Johns Hopkins University School of Medicine, Kennedy Krieger Institute, Baltimore, Maryland, Kathleen B. Schwarz, M.D., Professor, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland; Director, Pediatric Liver Center, Department of Pediatrics, Johns Hopkins Children's Center, Baltimore, Maryland
- Edited by Frederick J. Suchy, Mount Sinai School of Medicine, New York, Ronald J. Sokol, University of Colorado, Denver, William F. Balistreri, University of Cincinnati
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- Book:
- Liver Disease in Children
- Published online:
- 18 December 2009
- Print publication:
- 07 May 2007, pp 840-857
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Summary
PEROXISOMAL STRUCTURE AND FUNCTIONS
General Aspects of Peroxisomes
Peroxisomes have the distinction of being the last true organelle discovered. They were first identified in renal proximal tubule cells by a Swedish graduate student in 1954. Initially called microbodies, these organelles were studied intensively by de Duve and coworkers. Because they contained enzymes that both produced (e.g., amino acid and urate oxidases) and degraded (e.g., catalase) hydrogen peroxide, de Duve and Baudhuin [1] proposed the name peroxisomes. Microbodies found in some lower organisms and plants were named for the specialized functions that they carry out. For example, glyoxysomes of fungi and plants contain the five enzymes of the glyoxylate cycle and glycosomes house the enzymes of glycolysis in trypanosomes [2, 3]. Peroxisomes have been found in essentially all plant and animal cells with the exception of mature erythrocytes, and they range in size from about 0.1 μm (microperoxisomes of intestine and brain) up to 1.0 μm (characteristic of hepatic and renal peroxisomes; range: 0.2–1.0 μm) [4] (Figure 35.1).
A single lipid bilayer comprises the peroxisomal membrane. The organelle's matrix is finely granular, but microcrystalline cores of urate oxidase are present in the hepatic peroxisomes of some species (e.g., rats). No cores are found in human peroxisomes because humans lack urate oxidase. Unlike chloroplasts and mitochondria, peroxisomes contain no DNA, although it has been speculated that all three organelles evolved from endosymbionts. Since discovery of peroxisomes, numerous membrane proteins and matrix enzymes have been identified.
Looking Backward, Looking Forward: MLA Members Speak
- April Alliston, Elizabeth Ammons, Jean Arnold, Nina Baym, Sandra L. Beckett, Peter G. Beidler, Roger A. Berger, Sandra Bermann, J.J. Wilson, Troy Boone, Alison Booth, Wayne C. Booth, James Phelan, Marie Borroff, Ihab Hassan, Ulrich Weisstein, Zack Bowen, Jill Campbell, Dan Campion, Jay Caplan, Maurice Charney, Beverly Lyon Clark, Robert A. Colby, Thomas C. Coleman III, Nicole Cooley, Richard Dellamora, Morris Dickstein, Terrell Dixon, Emory Elliott, Caryl Emerson, Ann W. Engar, Lars Engle, Kai Hammermeister, N. N. Feltes, Mary Anne Ferguson, Annie Finch, Shelley Fisher Fishkin, Jerry Aline Flieger, Norman Friedman, Rosemarie Garland-Thomson, Sandra M. Gilbert, Laurie Grobman, George Guida, Liselotte Gumpel, R. K. Gupta, Florence Howe, Cathy L. Jrade, Richard A. Kaye, Calhoun Winton, Murray Krieger, Robert Langbaum, Richard A. Lanham, Marilee Lindemann, Paul Michael Lützeler, Thomas J. Lynn, Juliet Flower MacCannell, Michelle A. Massé, Irving Massey, Georges May, Christian W. Hallstein, Gita May, Lucy McDiarmid, Ellen Messer-Davidow, Koritha Mitchell, Robin Smiles, Kenyatta Albeny, George Monteiro, Joel Myerson, Alan Nadel, Ashton Nichols, Jeffrey Nishimura, Neal Oxenhandler, David Palumbo-Liu, Vincent P. Pecora, David Porter, Nancy Potter, Ronald C. Rosbottom, Elias L. Rivers, Gerhard F. Strasser, J. L. Styan, Marianna De Marco Torgovnick, Gary Totten, David van Leer, Asha Varadharajan, Orrin N. C. Wang, Sharon Willis, Louise E. Wright, Donald A. Yates, Takayuki Yokota-Murakami, Richard E. Zeikowitz, Angelika Bammer, Dale Bauer, Karl Beckson, Betsy A. Bowen, Stacey Donohue, Sheila Emerson, Gwendolyn Audrey Foster, Jay L. Halio, Karl Kroeber, Terence Hawkes, William B. Hunter, Mary Jambus, Willard F. King, Nancy K. Miller, Jody Norton, Ann Pellegrini, S. P. Rosenbaum, Lorie Roth, Robert Scholes, Joanne Shattock, Rosemary T. VanArsdel, Alfred Bendixen, Alarma Kathleen Brown, Michael J. Kiskis, Debra A. Castillo, Rey Chow, John F. Crossen, Robert F. Fleissner, Regenia Gagnier, Nicholas Howe, M. Thomas Inge, Frank Mehring, Hyungji Park, Jahan Ramazani, Kenneth M. Roemer, Deborah D. Rogers, A. LaVonne Brown Ruoff, Regina M. Schwartz, John T. Shawcross, Brenda R. Silver, Andrew von Hendy, Virginia Wright Wexman, Britta Zangen, A. Owen Aldridge, Paula R. Backscheider, Roland Bartel, E. M. Forster, Milton Birnbaum, Jonathan Bishop, Crystal Downing, Frank H. Ellis, Roberto Forns-Broggi, James R. Giles, Mary E. Giles, Susan Blair Green, Madelyn Gutwirth, Constance B. Hieatt, Titi Adepitan, Edgar C. Knowlton, Jr., Emanuel Mussman, Sally Todd Nelson, Robert O. Preyer, David Diego Rodriguez, Guy Stern, James Thorpe, Robert J. Wilson, Rebecca S. Beal, Joyce Simutis, Betsy Bowden, Sara Cooper, Wheeler Winston Dixon, Tarek el Ariss, Richard Jewell, John W. Kronik, Wendy Martin, Stuart Y. McDougal, Hugo Méndez-Ramírez, Ivy Schweitzer, Armand E. Singer, G. Thomas Tanselle, Tom Bishop, Mary Ann Caws, Marcel Gutwirth, Christophe Ippolito, Lawrence D. Kritzman, James Longenbach, Tim McCracken, Wolfe S. Molitor, Diane Quantic, Gregory Rabassa, Ellen M. Tsagaris, Anthony C. Yu, Betty Jean Craige, Wendell V. Harris, J. Hillis Miller, Jesse G. Swan, Helene Zimmer-Loew, Peter Berek, James Chandler, Hanna K. Charney, Philip Cohen, Judith Fetterley, Herbert Lindenberger, Julia Reinhard Lupton, Maximillian E. Novak, Richard Ohmann, Marjorie Perloff, Mark Reynolds, James Sledd, Harriet Turner, Marie Umeh, Flavia Aloya, Regina Barreca, Konrad Bieber, Ellis Hanson, William J. Hyde, Holly A. Laird, David Leverenz, Allen Michie, J. Wesley Miller, Marvin Rosenberg, Daniel R. Schwarz, Elizabeth Welt Trahan, Jean Fagan Yellin
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- Journal:
- PMLA / Publications of the Modern Language Association of America / Volume 115 / Issue 7 / December 2000
- Published online by Cambridge University Press:
- 23 October 2020, pp. 1986-2078
- Print publication:
- December 2000
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