Advertisement
JBC

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Originally published In Press as doi:10.1074/jbc.M212715200 on March 6, 2003

J. Biol. Chem., Vol. 278, Issue 20, 18491-18498, May 16, 2003
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow All Versions of this Article:
278/20/18491    most recent
M212715200v1
Right arrow Submit a Letter to Editor
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrowRequest Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Kozel, B. A.
Right arrow Articles by Mecham, R. P.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Kozel, B. A.
Right arrow Articles by Mecham, R. P.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?

Domains in Tropoelastin That Mediate Elastin Deposition in Vitro and in Vivo*

Beth A. KozelDagger , Hiroshi Wachi§, Elaine C. Davis, and Robert P. MechamDagger ||

From the Dagger  Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, Missouri 63110, the § Department of Clinical Chemistry, Hoshi University School of Pharmacy, Tokyo 142-850, Japan, and the  Department of Anatomy and Cell Biology, McGill University, Montreal, Quebec H3A 2B2, Canada

Elastic fiber assembly is a complicated process involving multiple different proteins and enzyme activities. However, the specific protein-protein interactions that facilitate elastin polymerization have not been defined. To identify domains in the tropoelastin molecule important for the assembly process, we utilized an in vitro assembly model to map sequences within tropoelastin that facilitate its association with fibrillin-containing microfibrils in the extracellular matrix. Our results show that an essential assembly domain is located in the C-terminal region of the molecule, encoded by exons 29-36. Fine mapping studies using an exon deletion strategy and synthetic peptides identified the hydrophobic sequence in exon 30 as a major functional element in this region and suggested that the assembly process is driven by the propensity of this sequence to form beta -sheet structure. Tropoelastin molecules lacking the C-terminal assembly domain expressed as transgenes in mice did not assemble nor did they interfere with assembly of full-length normal mouse elastin. In addition to providing important information about elastin assembly in general, the results of this study suggest how removal or alteration of the C terminus through stop or frameshift mutations might contribute to the elastin-related diseases supravalvular aortic stenosis and cutis laxa.

* This work was supported by Grants HL53325, HL62295, and HL61006 from the National Institutes of Health and by a grant from the National Marfan Foundation.The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

|| To whom correspondence should be addressed: Dept. of Cell Biology and Physiology, Washington University School of Medicine, Campus Box 8228, 660 South Euclid Ave., St. Louis, MO 63110. Tel.: 314-362-2254; Fax: 314-362-2252; E-mail: bmecham@cellbiology.wustl.edu.

Copyright © 2003 by The American Society for Biochemistry and Molecular Biology, Inc.
Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
 J. Biol. Chem.Home page
J. S. Weinbaum, T. J. Broekelmann, R. A. Pierce, C. C. Werneck, F. Segade, C. S. Craft, R. H. Knutsen, and R. P. Mecham
Deficiency in Microfibril-associated Glycoprotein-1 Leads to Complex Phenotypes in Multiple Organ Systems
J. Biol. Chem., September 12, 2008; 283(37): 25533 - 25543.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
L. L. del Mercato, P. P. Pompa, G. Maruccio, A. D. Torre, S. Sabella, A. M. Tamburro, R. Cingolani, and R. Rinaldi
Charge transport and intrinsic fluorescence in amyloid-like fibrils
PNAS, November 13, 2007; 104(46): 18019 - 18024.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
E. Hirano, R. H. Knutsen, H. Sugitani, C. H. Ciliberto, and R. P. Mecham
Functional Rescue of Elastin Insufficiency in Mice by the Human Elastin Gene: Implications for Mouse Models of Human Disease
Circ. Res., August 31, 2007; 101(5): 523 - 531.
[Abstract] [Full Text] [PDF]

Home page
 Proc Am Thorac SocHome page
A. Shifren and R. P. Mecham
The stumbling block in lung repair of emphysema: elastic fiber assembly.
Proceedings of the ATS, July 1, 2006; 3(5): 428 - 433.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Cell. Biol.Home page
P. J. McLaughlin, Q. Chen, M. Horiguchi, B. C. Starcher, J. B. Stanton, T. J. Broekelmann, A. D. Marmorstein, B. McKay, R. Mecham, T. Nakamura, et al.
Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice.
Mol. Cell. Biol., March 1, 2006; 26(5): 1700 - 1709.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
L. Thomassin, C. C. Werneck, T. J. Broekelmann, C. Gleyzal, I. K. Hornstra, R. P. Mecham, and P. Sommer
The Pro-regions of Lysyl Oxidase and Lysyl Oxidase-like 1 Are Required for Deposition onto Elastic Fibers
J. Biol. Chem., December 30, 2005; 280(52): 42848 - 42855.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
T. J. Broekelmann, B. A. Kozel, H. Ishibashi, C. C. Werneck, F. W. Keeley, L. Zhang, and R. P. Mecham
Tropoelastin Interacts with Cell-surface Glycosaminoglycans via Its COOH-terminal Domain
J. Biol. Chem., December 9, 2005; 280(49): 40939 - 40947.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Cell Mol. Bio.Home page
C. M. Kelleher, E. K. Silverman, T. Broekelmann, A. A. Litonjua, M. Hernandez, J. S. Sylvia, J. Stoler, J. J. Reilly, H. A. Chapman, F. E. Speizer, et al.
A Functional Mutation in the Terminal Exon of Elastin in Severe, Early-Onset Chronic Obstructive Pulmonary Disease
Am. J. Respir. Cell Mol. Biol., October 1, 2005; 33(4): 355 - 362.
[Abstract] [Full Text] [PDF]

Home page
 J. Appl. Physiol.Home page
B. Suki, S. Ito, D. Stamenovic, K. R. Lutchen, and E. P. Ingenito
Biomechanics of the lung parenchyma: critical roles of collagen and mechanical forces
J Appl Physiol, May 1, 2005; 98(5): 1892 - 1899.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
A. M. Tamburro, A. Pepe, B. Bochicchio, D. Quaglino, and I. P. Ronchetti
Supramolecular Amyloid-like Assembly of the Polypeptide Sequence Coded by Exon 30 of Human Tropoelastin
J. Biol. Chem., January 28, 2005; 280(4): 2682 - 2690.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
M. Miao, C. M. Bellingham, R. J. Stahl, E. E. Sitarz, C. J. Lane, and F. W. Keeley
Sequence and Structure Determinants for the Self-aggregation of Recombinant Polypeptides Modeled after Human Elastin
J. Biol. Chem., December 5, 2003; 278(49): 48553 - 48562.
[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 
Copyright © 2003 by the American Society for Biochemistry and Molecular Biology.
Advertisement
spacer
Advertisement
Advertisement