Universität zu Köln

Chiquet, Matthias ORCID: 0000-0001-7812-4853, Birk, David E., Boennemann, Carsten G. and Koch, Manuel ORCID: 0000-0002-2962-7814 (2014). Collagen XII: Protecting bone and muscle integrity by organizing collagen fibrils. Int. J. Biochem. Cell Biol., 53. S. 51 - 55. OXFORD: PERGAMON-ELSEVIER SCIENCE LTD. ISSN 1878-5875

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Abstract

Collagen XII, largest member of the fibril-associated collagens with interrupted triple helix (FACIT) family, assembles from three identical alpha-chains encoded by the COL12A1 gene. The molecule consists of three threadlike N-terminal noncollagenous NC3 domains, joined by disulfide bonds and a short interrupted collagen triple helix toward the C-terminus. Splice variants differ considerably in size and properties: small collagen XIIB (220 kDa subunit) is similar to collagen XIV, whereas collagen XIIA (350 kDa) has a much larger NC3 domain carrying glycosaminoglycan chains. Collagen XII binds to collagen I-containing fibrils via its collagenous domain, whereas its large noncollagenous arms interact with other matrix proteins such as tenascin-X. In dense connective tissues and bone, collagen XII is thought to regulate organization and mechanical properties of collagen fibril bundles. Accordingly, recent findings show that collagen XII mutations cause Ehlers-Danlos/myopathy overlap syndrome associated with skeletal abnormalities and muscle weakness in mice and humans. (C) 2014 Elsevier Ltd. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Chiquet, Matthias UNSPECIFIED orcid.org/0000-0001-7812-4853 UNSPECIFIED Birk, David E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Boennemann, Carsten G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Koch, Manuel UNSPECIFIED orcid.org/0000-0002-2962-7814 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-432635
DOI:	10.1016/j.biocel.2014.04.020
Journal or Publication Title:	Int. J. Biochem. Cell Biol.
Volume:	53
Page Range:	S. 51 - 55
Date:	2014
Publisher:	PERGAMON-ELSEVIER SCIENCE LTD
Place of Publication:	OXFORD
ISSN:	1878-5875
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language 2 SPLICE VARIANTS; DIFFERENTIAL EXPRESSION; DOMAIN; FORM; SKIN; CARTILAGE; MOLECULE; TENDON; CHICK; IX Multiple languages Biochemistry & Molecular Biology; Cell Biology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/43263