| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
(Received for publication, March 7, 1996, and in revised form, April 29, 1996)
From the Department of Internal Medicine, University of Texas
Southwestern Medical Center, Dallas, Texas 75235-8593
Palmitoyl-protein thioesterase is a newly
described long chain fatty-acid hydrolase that removes fatty acyl
groups from modified cysteines in proteins. We have recently
identified palmitoyl-protein thioesterase as the defective enzyme in
the recessive hereditary neurological degenerative disorder infantile
neuronal ceroid lipofuscinosis (Vesa, J., Hellsten, E., Verkruyse, L. A., Camp, L. A., Rapola, J., Santavuori, P., Hofmann, S. L., and
Peltonen, L. (1995) Nature 376, 584-587). A defect in a
lysosomal enzyme had been postulated for the disease, but until
recently, the relevant defective lysosomal enzyme had not been
identified.
In this paper, we present evidence for the lysosomal localization of
palmitoyl-protein thioesterase. We show that COS cells take up
exogenously supplied palmitoyl-protein thioesterase intracellularly and
that the cellular uptake is blocked by mannose 6-phosphate, a hallmark
of lysosomal enzyme trafficking. The enzyme contains endoglycosidase
H-sensitive oligosaccharides that contain phosphate groups.
Furthermore, palmitoyl-protein thioesterase cosediments with lysosomal
enzyme markers by Percoll density gradient centrifugation.
Interestingly, the pH optimum for the enzyme is in the neutral range, a
property shared by two other lysosomal enzymes that remove
post-translational protein modifications. These findings suggest that
palmitoyl-protein thioesterase is a lysosomal enzyme and that infantile
neuronal ceroid lipofuscinosis is properly classified as a lysosomal
storage disorder.
Volume 271, Number 26,
Issue of June 28, 1996
pp. 15831-15836
©1996 by The American Society for Biochemistry and Molecular Biology, Inc.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  N. A. Pfender, S. Grosch, G. Roussel, M. Koch, E. Trifilieff, and J. M. Greer Route of Uptake of Palmitoylated Encephalitogenic Peptides of Myelin Proteolipid Protein by Antigen-Presenting Cells: Importance of the Type of Bond between Lipid Chain and Peptide and Relevance to Autoimmunity J. Immunol., February 1, 2008; 180(3): 1398 - 1404. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Buff, A. C. Smith, and C. A. Korey Genetic Modifiers of Drosophila Palmitoyl-Protein Thioesterase 1-Induced Degeneration Genetics, May 1, 2007; 176(1): 209 - 220. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J.-Y. Lu and S. L. Hofmann Thematic review series: Lipid Posttranslational Modifications. Lysosomal metabolism of lipid-modified proteins J. Lipid Res., July 1, 2006; 47(7): 1352 - 1357. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Roy, S. Plowman, B. Rotblat, I. A. Prior, C. Muncke, S. Grainger, R. G. Parton, Y. I. Henis, Y. Kloog, and J. F. Hancock Individual Palmitoyl Residues Serve Distinct Roles in H-Ras Trafficking, Microlocalization, and Signaling Mol. Cell. Biol., August 1, 2005; 25(15): 6722 - 6733. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Stockli and J. Rohrer The Palmitoyltransferase of the Cation-dependent Mannose 6-Phosphate Receptor Cycles between the Plasma Membrane and Endosomes Mol. Biol. Cell, June 1, 2004; 15(6): 2617 - 2626. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. K. Cho and S. L. Hofmann pdf1, a Palmitoyl Protein Thioesterase 1 Ortholog in Schizosaccharomyces pombe: a Yeast Model of Infantile Batten Disease Eukaryot. Cell, April 1, 2004; 3(2): 302 - 310. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. S. Rush, S. K. Cho, S. Jiang, S. L. Hofmann, and C. J. Waechter Identification and Characterization of a cDNA Encoding a Dolichyl Pyrophosphate Phosphatase Located in the Endoplasmic Reticulum of Mammalian Cells J. Biol. Chem., November 15, 2002; 277(47): 45226 - 45234. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Singh, L. Young, D. J. Handelsman, and Q. Dong Prostate Epithelial Expression of a Novel Androgen Target Gene J Androl, September 1, 2002; 23(5): 652 - 660. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Vesa, M. H. Chin, K. Oelgeschlager, J. Isosomppi, E. C. DellAngelica, A. Jalanko, and L. Peltonen Neuronal Ceroid Lipofuscinoses Are Connected at Molecular Level: Interaction of CLN5 Protein with CLN2 and CLN3 Mol. Biol. Cell, July 1, 2002; 13(7): 2410 - 2420. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Isosomppi, J. Vesa, A. Jalanko, and L. Peltonen Lysosomal localization of the neuronal ceroid lipofuscinosis CLN5 protein Hum. Mol. Genet., April 15, 2002; 11(8): 885 - 891. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Lonnqvist, S. L. Vanhanen, K. Vettenranta, T. Autti, J. Rapola, P. Santavuori, and U.M. Saarinen-Pihkala Hematopoietic stem cell transplantation in infantile neuronal ceroid lipofuscinosis Neurology, October 23, 2001; 57(8): 1411 - 1416. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. K. Das, J.-Y. Lu, and S. L. Hofmann Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis Hum. Mol. Genet., June 1, 2001; 10(13): 1431 - 1439. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Lehtovirta, A. Kyttala, E.-L. Eskelinen, M. Hess, O. Heinonen, and A. Jalanko Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL) Hum. Mol. Genet., January 1, 2001; 10(1): 69 - 75. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Lonka, A. Kyttala, S. Ranta, A. Jalanko, and A.-E. Lehesjoki The neuronal ceroid lipofuscinosis CLN8 membrane protein is a resident of the endoplasmic reticulum Hum. Mol. Genet., July 1, 2000; 9(11): 1691 - 1697. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Peltonen, A. Jalanko, and T. Varilo Molecular genetics of the Finnishdisease heritage Hum. Mol. Genet., September 1, 1999; 8(10): 1913 - 1923. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. Poupon, B. Begue, J. Gagnon, A. Dautry-Varsat, N. Cerf-Bensussan, and A. Benmerah Molecular Cloning and Characterization of MT-ACT48, a Novel Mitochondrial Acyl-CoA Thioesterase J. Biol. Chem., July 2, 1999; 274(27): 19188 - 19194. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y V Voznyi, J L M Keulemans, G M S Mancini, C E Catsman-Berrevoets, E Young, B Winchester, W J Kleijer, and O P van Diggelen A new simple enzyme assay for pre- and postnatal diagnosis of infantile neuronal ceroid lipofuscinosis (INCL) and its variants J. Med. Genet., June 1, 1999; 36(6): 471 - 474. [Abstract] [Full Text]
|
|
|
|
 |
|
 D. J. Hackam, O. D. Rotstein, W.-j. Zhang, S. Gruenheid, P. Gros, and S. Grinstein Host Resistance to Intracellular Infection: Mutation of Natural Resistance-associated Macrophage Protein 1 (Nramp1) Impairs Phagosomal Acidification J. Exp. Med., July 20, 1998; 188(2): 351 - 364. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. A. Duncan and A. G. Gilman A Cytoplasmic Acyl-Protein Thioesterase That Removes Palmitate from G Protein alpha Subunits and p21RAS J. Biol. Chem., June 19, 1998; 273(25): 15830 - 15837. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. A. Soyombo and S. L. Hofmann Molecular Cloning and Expression of Palmitoyl-protein Thioesterase 2 (PPT2), a Homolog of Lysosomal Palmitoyl-protein Thioesterase with a Distinct Substrate Specificity J. Biol. Chem., October 24, 1997; 272(43): 27456 - 27463. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. A. Chen and D. R. Manning Regulation of Galpha i Palmitoylation by Activation of the 5-Hydroxytryptamine-1A Receptor J. Biol. Chem., July 28, 2000; 275(31): 23516 - 23522. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Sabri, G. Bare, P. Jacques, A. Jabrane, M. Ongena, J. C. Van Heugen, B. Devreese, and P. Thonart Influence of Moderate Temperatures on Myristoyl-CoA Metabolism and Acyl-CoA Thioesterase Activity in the Psychrophilic Antarctic Yeast Rhodotorula aurantiaca J. Biol. Chem., April 13, 2001; 276(16): 12691 - 12696. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. J. Bellizzi III, J. Widom, C. Kemp, J.-Y. Lu, A. K. Das, S. L. Hofmann, and J. Clardy The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis PNAS, April 25, 2000; 97(9): 4573 - 4578. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |
