Domains 16 and 17 of tropoelastin in elastic fibre formation.

Domains 16 and 17 of tropoelastin in elastic fibre formation. Research Abstract Details 

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Domains 16 and 17 of tropoelastin in elastic fibre formation. Abstract Text:

Hiroshi Wachi,Fumiaki Sato,Junji Nakazawa,Risa Nonaka,Zoltan Szabo,Zsolt Urban,Takuo Yasunaga,Iori Maeda,Koji Okamoto,Barry C Starcher,Dean Y Li,Robert P Mecham,Yoshiyuki Seyama,

Naturally occurring mutations are useful in identifying domains that are important for protein function. We studied a mutation in the elastin gene, 800-3G>C, a common disease allele for SVAS (supravalvular aortic stenosis). We showed in primary skin fibroblasts from two different SVAS families that this mutation causes skipping of exons 16-17 and results in a stable mRNA. Tropoelastin lacking domains 16-17 (Delta16-17) was synthesized efficiently and secreted by transfected retinal pigment epithelium cells, but showed the deficient deposition into the extracellular matrix compared with normal as demonstrated by immunofluorescent staining and desmosine assays. Solid-phase binding assays indicated normal molecular interaction of Delta16-17 with fibrillin-1 and fibulin-5. However, self-association of Delta16-17 was diminished as shown by an elevated coacervation temperature. Moreover, negative staining electron microscopy confirmed that Delta16-17 was deficient in forming fibrillar polymers. Domain 16 has high homology with domain 30, which can form a beta-sheet structure facilitating fibre formation. Taken together, we conclude that domains 16-17 are important for self-association of tropoelastin and elastic fibre formation. This study is the first to discover that domains of elastin play an essential role in elastic fibre formation by facilitating homotypic interactions.

Domains 16 and 17 of tropoelastin in elastic fibre formation. Publishing Authors By Initials

H Wachi,F Sato,J Nakazawa,R Nonaka,Z Szabo,Z Urban,T Yasunaga,I Maeda,K Okamoto,BC Starcher,DY Li,RP Mecham,Y Seyama,

For similar proteins: protein precursors: tropoelastin research abstracts see: proteins: protein precursors: tropoelastin research

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Domains 16 and 17 of tropoelastin in elastic fibre formation. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: The Biochemical journal

VOLUME: 402

Page Numbers: 63-70

Journal Abbreviation: Biochem. J.

ISSN: 1470-8728

DAY: 15

MONTH: Feb

YEAR: 2007

Domains 16 and 17 of tropoelastin in elastic fibre formation. Information

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LANGUAGE: eng

NlmUniqueID: 2984726

Domains 16 and 17 of tropoelastin in elastic fibre formation. Keywords Mesh Terms:

KEYWORDS: Tropoelastin

MESH TERMS: metabolism

Chemical & Substance for Abstract: Domains 16 and 17 of tropoelastin in elastic fibre formation. Information

Substance Name: Tropoelastin

Registry Number: 0

Grant and Affiliation Information for Domains 16 and 17 of tropoelastin in elastic fibre formation.

AFFILIATION: Department of Clinical Chemistry, Hoshi University School of Pharmacy and Pharmaceutical Sciences, 2-4-41 Ebara, Shinagawa-ku, Tokyo 142-8501, Japan. wachi@hoshi.ac.jp

Country: England

AGENCY: United States NHLBI

GRANT: HL073703

ACRONYM: HL

MEDLINETA: Biochem J

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