Universität zu Köln

Patel, Trushar R. ORCID: 0000-0003-0627-2923, Nikodemus, Denise ORCID: 0000-0003-4492-817X, Besong, Tabot M. D., Reuten, Raphael ORCID: 0000-0002-9434-4108, Meier, Markus ORCID: 0000-0003-1068-746X, Harding, Stephen E. ORCID: 0000-0002-7798-9692, Winzor, Donald J., Koch, Manuel and Stetefeld, Joerg ORCID: 0000-0003-1478-3248 (2016). Biophysical analysis of a lethal laminin alpha-1 mutation reveals altered self-interaction. Matrix Biol., 49. S. 93 - 106. AMSTERDAM: ELSEVIER SCIENCE BV. ISSN 1569-1802

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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 alpha, one beta, and one gamma 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 alpha-1 (hLM alpha-1 N), alpha-5 (hLM alpha-5 N) and beta-3 (hLM beta-3 N) originating from the short arms of the human laminin alpha beta gamma heterotrimer. Corresponding studies of the hLM alpha-1 N 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. (C) 2015 Elsevier B.V. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Patel, Trushar R. UNSPECIFIED orcid.org/0000-0003-0627-2923 UNSPECIFIED Nikodemus, Denise UNSPECIFIED orcid.org/0000-0003-4492-817X UNSPECIFIED Besong, Tabot M. D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Reuten, Raphael UNSPECIFIED orcid.org/0000-0002-9434-4108 UNSPECIFIED Meier, Markus UNSPECIFIED orcid.org/0000-0003-1068-746X UNSPECIFIED Harding, Stephen E. UNSPECIFIED orcid.org/0000-0002-7798-9692 UNSPECIFIED Winzor, Donald J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Koch, Manuel UNSPECIFIED UNSPECIFIED UNSPECIFIED Stetefeld, Joerg UNSPECIFIED orcid.org/0000-0003-1478-3248 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-293022
DOI:	10.1016/j.matbio.2015.06.005
Journal or Publication Title:	Matrix Biol.
Volume:	49
Page Range:	S. 93 - 106
Date:	2016
Publisher:	ELSEVIER SCIENCE BV
Place of Publication:	AMSTERDAM
ISSN:	1569-1802
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language COILED-COIL DOMAIN; BETA-3 EXPRESSION; CHAIN; SEDIMENTATION; MIGRATION; COMPLEX; GENE; FORM Multiple languages Biochemistry & Molecular Biology; Cell Biology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/29302