|
|
|
|
|
|
|
|
|||||
| Home Help Feedback Subscriptions Archive Search Table of Contents | |||||
Journal of Cell Science, Vol 112, Issue 10 1579-1590, Copyright © 1999 by Company of Biologists
JOURNAL ARTICLES |
CC Chang, S South, D Warren, J Jones, AB Moser, HW Moser and SJ Gould
The Department of Biological Chemistry, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Zellweger syndrome and related disorders represent a group of lethal, genetically heterogeneous diseases. These peroxisome biogenesis disorders (PBDs) are characterized by defective peroxisomal matrix protein import and comprise at least 10 complementation groups. The genes defective in seven of these groups and more than 90% of PBD patients are now known. Here we examine the distribution of peroxisomal membrane proteins in fibroblasts from PBD patients representing the seven complementation groups for which the mutant gene is known. Peroxisomes were detected in all PBD cells, indicating that the ability to form a minimal peroxisomal structure is not blocked in these mutants. We also observed that peroxisome abundance was reduced fivefold in PBD cells that are defective in the PEX1, PEX5, PEX12, PEX6, PEX10, and PEX2 genes. These cell lines all display a defect in the import of proteins with the type-1 peroxisomal targeting signal (PTS1). In contrast, peroxisome abundance was unaffected in cells that are mutated in PEX7 and are defective only in the import of proteins with the type-2 peroxisomal targeting signal. Interestingly, a fivefold reduction in peroxisome abundance was also observed for cells lacking either of two PTS1-targeted peroxisomal beta-oxidation enzymes, acyl-CoA oxidase and 2-enoyl-CoA hydratase/D-3-hydroxyacyl-CoA dehydrogenase. These results indicate that reduced peroxisome abundance in PBD cells may be caused by their inability to import these PTS1-containing enzymes. Furthermore, the fact that peroxisome abundance is influenced by peroxisomal 105-oxidation activities suggests that there may be metabolic control of peroxisome abundance.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati 
Twitter What's this?
This article has been cited by other articles:
|
|
 |
|
  K. Piekarska, G. Hardy, E. Mol, J. van den Burg, K. Strijbis, C. van Roermund, M. van den Berg, and B. Distel The activity of the glyoxylate cycle in peroxisomes of Candida albicans depends on a functional {beta}-oxidation pathway: evidence for reduced metabolite transport across the peroxisomal membrane Microbiology, October 1, 2008; 154(10): 3061 - 3072. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. A. Saleem, B. Knoblach, F. D. Mast, J. J. Smith, J. Boyle, C. M. Dobson, R. Long-O'Donnell, R. A. Rachubinski, and J. D. Aitchison Genome-wide analysis of signaling networks regulating fatty acid-induced gene expression and organelle biogenesis J. Cell Biol., April 21, 2008; 181(2): 281 - 292. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Kashiwayama, K. Asahina, M. Morita, and T. Imanaka Hydrophobic Regions Adjacent to Transmembrane Domains 1 and 5 Are Important for the Targeting of the 70-kDa Peroxisomal Membrane Protein J. Biol. Chem., November 16, 2007; 282(46): 33831 - 33844. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Orth, S. Reumann, X. Zhang, J. Fan, D. Wenzel, S. Quan, and J. Hu The PEROXIN11 Protein Family Controls Peroxisome Proliferation in Arabidopsis PLANT CELL, January 1, 2007; 19(1): 333 - 350. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. J. Vizeacoumar, W. N. Vreden, M. Fagarasanu, G. A. Eitzen, J. D. Aitchison, and R. A. Rachubinski The Dynamin-like Protein Vps1p of the Yeast Saccharomyces cerevisiae Associates with Peroxisomes in a Pex19p-dependent Manner J. Biol. Chem., May 5, 2006; 281(18): 12817 - 12823. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Nguyen, J. Bjorkman, B. C. Paton, and D. I. Crane Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance J. Cell Sci., February 15, 2006; 119(4): 636 - 645. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H Rosewich, A Ohlenbusch, and J Gartner Genetic and clinical aspects of Zellweger spectrum patients with PEX1 mutations J. Med. Genet., September 1, 2005; 42(9): e58 - e58. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Fan, S. Quan, T. Orth, C. Awai, J. Chory, and J. Hu The Arabidopsis PEX12 Gene Is Required for Peroxisome Biogenesis and Is Essential for Development Plant Physiology, September 1, 2005; 139(1): 231 - 239. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Koch, G. Schneider, G. H. Luers, and M. Schrader Peroxisome elongation and constriction but not fission can occur independently of dynamin-like protein 1 J. Cell Sci., September 1, 2004; 117(17): 3995 - 4006. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. I. Petriv and R. A. Rachubinski Lack of Peroxisomal Catalase Causes a Progeric Phenotype in Caenorhabditis elegans J. Biol. Chem., May 7, 2004; 279(19): 19996 - 20001. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. Fang, J. C. Morrell, J. M. Jones, and S. J. Gould PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins J. Cell Biol., March 15, 2004; 164(6): 863 - 875. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. K. Zolman and B. Bartel An Arabidopsis indole-3-butyric acid-response mutant defective in PEROXIN6, an apparent ATPase implicated in peroxisomal function PNAS, February 10, 2004; 101(6): 1786 - 1791. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Shimizu, A. Takeshita, T. Tsukamoto, F. J. Gonzalez, and T. Osumi Tissue-Selective, Bidirectional Regulation of PEX11{alpha} and Perilipin Genes through a Common Peroxisome Proliferator Response Element Mol. Cell. Biol., February 1, 2004; 24(3): 1313 - 1323. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M. Jones, J. C. Morrell, and S. J. Gould PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins J. Cell Biol., January 5, 2004; 164(1): 57 - 67. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Rottensteiner, K. Stein, E. Sonnenhol, and R. Erdmann Conserved Function of Pex11p and the Novel Pex25p and Pex27p in Peroxisome Biogenesis Mol. Biol. Cell, October 1, 2003; 14(10): 4316 - 4328. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Guo, Y. Y. Kit, J.-M. Nicaud, M.-T. Le Dall, S. K. Sears, H. Vali, H. Chan, R. A. Rachubinski, and V. I. Titorenko Peroxisome division in the yeast Yarrowia lipolytica is regulated by a signal from inside the peroxisome J. Cell Biol., September 29, 2003; 162(7): 1255 - 1266. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. L. Rylott, C. A. Rogers, A. D. Gilday, T. Edgell, T. R. Larson, and I. A Graham Arabidopsis Mutants in Short- and Medium-chain Acyl-CoA Oxidase Activities Accumulate Acyl-CoAs and Reveal That Fatty Acid {beta}-Oxidation Is Essential for Embryo Development J. Biol. Chem., June 6, 2003; 278(24): 21370 - 21377. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
X. Li and S. J. Gould The Dynamin-like GTPase DLP1 Is Essential for Peroxisome Division and Is Recruited to Peroxisomes in Part by PEX11 J. Biol. Chem., May 2, 2003; 278(19): 17012 - 17020. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. J. Vizeacoumar, J. C. Torres-Guzman, Y. Y. C. Tam, J. D. Aitchison, and R. A. Rachubinski YHR150w and YDR479c encode peroxisomal integral membrane proteins involved in the regulation of peroxisome number, size, and distribution in Saccharomyces cerevisiae J. Cell Biol., April 28, 2003; 161(2): 321 - 332. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. A. Bascom, H. Chan, and R. A. Rachubinski Peroxisome Biogenesis Occurs in an Unsynchronized Manner in Close Association with the Endoplasmic Reticulum in Temperature-sensitive Yarrowia lipolytica Pex3p Mutants Mol. Biol. Cell, March 1, 2003; 14(3): 939 - 957. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
X. Li, E. Baumgart, G.-X. Dong, J. C. Morrell, G. Jimenez-Sanchez, D. Valle, K. D. Smith, and S. J. Gould PEX11{alpha} Is Required for Peroxisome Proliferation in Response to 4-Phenylbutyrate but Is Dispensable for Peroxisome Proliferator-Activated Receptor Alpha-Mediated Peroxisome Proliferation Mol. Cell. Biol., December 1, 2002; 22(23): 8226 - 8240. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. I. Petriv, D. B. Pilgrim, R. A. Rachubinski, and V. I. Titorenko RNA interference of peroxisome-related genes in C. elegans: a new model for human peroxisomal disorders Physiol Genomics, August 14, 2002; 10(2): 79 - 91. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Ruprich-Robert, V. Berteaux-Lecellier, D. Zickler, A. Panvier-Adoutte, and M. Picard Identification of Six Loci in Which Mutations Partially Restore Peroxisome Biogenesis and/or Alleviate the Metabolic Defect of pex2 Mutants in Podospora Genetics, July 1, 2002; 161(3): 1089 - 1099. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. C. Harper, S. T. South, J. M. McCaffery, and S. J. Gould Peroxisomal Membrane Protein Import Does Not Require Pex17p J. Biol. Chem., May 3, 2002; 277(19): 16498 - 16504. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Koenig, C. Araya, C. Skorin, C. Valencia, A. Toro, F. Leighton, and M. J. Santos Cytochemical and Biochemical Demonstration of an ATPase in Membranes of Human Peroxisomes J. Histochem. Cytochem., March 1, 2002; 50(3): 405 - 414. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
X. Li and S. J. Gould PEX11 promotes peroxisome division independently of peroxisome metabolism J. Cell Biol., February 18, 2002; 156(4): 643 - 651. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Dodt, D. Warren, E. Becker, P. Rehling, and S. J. Gould Domain Mapping of Human PEX5 Reveals Functional and Structural Similarities to Saccharomyces cerevisiae Pex18p and Pex21p J. Biol. Chem., November 2, 2001; 276(45): 41769 - 41781. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M. Jones, J. C. Morrell, and S. J. Gould Multiple Distinct Targeting Signals in Integral Peroxisomal Membrane Proteins J. Cell Biol., June 4, 2001; 153(6): 1141 - 1150. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. M. Olivier, W. Kovacs, K. Masuda, G.-A. Keller, and S. K. Krisans Identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes: AA-CoA thiolase, HMG-CoA synthase, MPPD, and FPP synthase J. Lipid Res., December 1, 2000; 41(12): 1921 - 1935. [Abstract] [Full Text]
|
|
|
|
|
|
C. W.T. van Roermund, H. F. Tabak, M. van den Berg, R. J.A. Wanders, and E. H. Hettema Pex11p Plays a Primary Role in Medium-Chain Fatty Acid Oxidation, a Process that Affects Peroxisome Number and Size in Saccharomyces cerevisiae J. Cell Biol., August 7, 2000; 150(3): 489 - 498. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. T. South, K. A. Sacksteder, X. Li, Y. Liu, and S. J. Gould Inhibitors of COPI and COPII Do Not Block PEX3-mediated Peroxisome Synthesis J. Cell Biol., June 26, 2000; 149(7): 1345 - 1360. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M. Jones, J. C. Morrell, and S. J. Gould Identification and Characterization of HAOX1, HAOX2, and HAOX3, Three Human Peroxisomal 2-Hydroxy Acid Oxidases J. Biol. Chem., April 21, 2000; 275(17): 12590 - 12597. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. A. Sacksteder, J. M. Jones, S. T. South, X. Li, Y. Liu, and S. J. Gould PEX19 Binds Multiple Peroxisomal Membrane Proteins, Is Predominantly Cytoplasmic, and Is Required for Peroxisome Membrane Synthesis J. Cell Biol., March 6, 2000; 148(5): 931 - 944. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Yamasaki, N. Hashiguchi, C. Fujiwara, T. Imanaka, T. Tsukamoto, and T. Osumi Formation of Peroxisomes from Peroxisomal Ghosts in a Peroxisome-deficient Mammalian Cell Mutant upon Complementation by Protein Microinjection J. Biol. Chem., December 10, 1999; 274(50): 35293 - 35296. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C.-C. Chang, D. S. Warren, K. A. Sacksteder, and S. J. Gould PEX12 Interacts with PEX5 and PEX10 and Acts Downstream of Receptor Docking in Peroxisomal Matrix Protein Import J. Cell Biol., November 15, 1999; 147(4): 761 - 774. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Gurvitz, J. K. Hiltunen, R. Erdmann, B. Hamilton, A. Hartig, H. Ruis, and H. Rottensteiner Saccharomyces cerevisiae Adr1p Governs Fatty Acid beta -Oxidation and Peroxisome Proliferation by Regulating POX1 and PEX11 J. Biol. Chem., August 17, 2001; 276(34): 31825 - 31830. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. J. Smith, T. W. Brown, G. A. Eitzen, and R. A. Rachubinski Regulation of Peroxisome Size and Number by Fatty Acid beta -Oxidation in the Yeast Yarrowia lipolytica J. Biol. Chem., June 23, 2000; 275(26): 20168 - 20178. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
X. Li and S. J. Gould PEX11 promotes peroxisome division independently of peroxisome metabolism J. Cell Biol., February 18, 2002; 156(4): 643 - 651. [Abstract] [Full Text] [PDF]
|
|
