Title |
Biophysical analysis of a lethal laminin alpha-1 mutation reveals altered self-interaction
|
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Published in |
Matrix Biology, July 2015
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DOI | 10.1016/j.matbio.2015.06.005 |
Pubmed ID | |
Authors |
Trushar R. Patel, Denise Nikodemus, Tabot M.D. Besong, Raphael Reuten, Markus Meier, Stephen E. Harding, Donald J. Winzor, Manuel Koch, Jörg Stetefeld |
Abstract |
Laminins are key basement membrane molecules that influence several biological activities and are linked to a number of diseases. They are secreted as heterotrimeric proteins consisting of one α, one β, and one γ chain, followed by their assembly into a polymer-like sheet at the basement membrane. Using sedimentation velocity, dynamic light scattering, and surface plasmon resonance experiments, we studied self-association of three laminin (LM) N-terminal fragments α-1 (hLM α-1N), α-5 (hLM α-5N) and β-3 (hLM β-3N) originating from the short arms of the human laminin αβγ heterotrimer. Corresponding studies of the hLM α-1N C49S mutant, equivalent to the larval lethal C56S mutant in zebrafish, have shown that this mutation causes enhanced self-association behavior, an observation that provides a plausible explanation for the inability of laminin bearing this mutation to fulfill functional roles in vivo, and hence for the deleterious pathological consequences of the mutation on lens function. |
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