Universität zu Köln

Becker, Ann-Kathrin A., Mikolajek, Halina, Paulsson, Mats, Wagener, Raimund and Werner, Joern M. (2014). A Structure of a Collagen VI VWA Domain Displays N and C Termini at Opposite Sides of the Protein. Structure, 22 (2). S. 199 - 209. CAMBRIDGE: CELL PRESS. ISSN 1878-4186

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Abstract

Von Willebrand factor A (VWA) domains are versatile protein interaction domains with N and C termini in close proximity placing spatial constraints on overall protein structure. The 1.2 angstrom crystal structures of a collagen VI VWA domain and a disease-causing point mutant show C-terminal extensions that place the N and C termini at opposite ends. This allows a beads-on-a-string arrangement of multiple VWA domains as observed for ten N-terminal domains of the collagen VI alpha 3 chain. The extension is linked to the core domain by a salt bridge and two hydrophobic patches. Comparison of the wild-type and a muscular dystrophy-associated mutant structure identifies a potential perturbation of a protein interaction interface and indeed, the secretion of mutant collagen VI tetramers is affected. Homology modeling is used to locate a number of disease-associated mutations and analyze their structural impact, which will allow mechanistic analysis of collagen-VI-associated muscular dystrophy phenotypes.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Becker, Ann-Kathrin A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mikolajek, Halina UNSPECIFIED UNSPECIFIED UNSPECIFIED Paulsson, Mats UNSPECIFIED UNSPECIFIED UNSPECIFIED Wagener, Raimund UNSPECIFIED UNSPECIFIED UNSPECIFIED Werner, Joern M. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-446034
DOI:	10.1016/j.str.2013.06.028
Journal or Publication Title:	Structure
Volume:	22
Number:	2
Page Range:	S. 199 - 209
Date:	2014
Publisher:	CELL PRESS
Place of Publication:	CAMBRIDGE
ISSN:	1878-4186
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language VON-WILLEBRAND-FACTOR; A-DOMAIN; ALPHA-3(VI) CHAIN; MICROFIBRIL FORMATION; MUTATION; MODEL; BINDING; CONFORMATION; ORGANIZATION; SECRETION Multiple languages Biochemistry & Molecular Biology; Biophysics; Cell Biology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/44603