AMACO is a component of the basement membrane-associated Fraser complex

B12 SENGLEB2 WAGENER/ PAULSSONpublicationsRichardson RJ, Gebauer JM, Zhang JL, Kobbe B, Keene DR, Karlsen KR, Richetti S, Wohl AP, Sengle G, Neiss WF, Paulsson M, Hammerschmidt M, Wagener R; J Invest Dermatol. 2014 May;134(5):1313-22. doi: 10.1038/jid.2013.492. Epub 2013 Nov 14.

Abstract Fraser syndrome (FS) is a phenotypically variable, autosomal recessive disorder characterized by cryptophthalmus, cutaneous syndactyly, and other malformations resulting from mutations in FRAS1,...   Read More

A mouse model for dominant collagen VI disorders: heterozygous deletion of Col6a3 Exon 16

B2 WAGENER/ PAULSSONpublications Pan TC, Zhang RZ, Arita M, Bogdanovich S, Adams SM, Gara SK, Wagener R, Khurana TS, Birk DE, Chu ML; J Biol Chem. 2014 Apr 11;289(15):10293-307. doi: 10.1074/jbc.M114.549311. Epub 2014 Feb 22.

Abstract   Dominant and recessive mutations in collagen VI genes, COL6A1, COL6A2, and COL6A3, cause a continuous spectrum of disorders characterized by muscle weakness...   Read More

A structure of a collagen VI VWA domain displays N and C termini at opposite sides of the protein

B2 WAGENER/ PAULSSONpublicationsBecker AK, Mikolajek H, Paulsson M, Wagener R, Werner JM; Structure. 2014 Feb 4;22(2):199-208. doi: 10.1016/j.str.2013.06.028. Epub 2013 Dec 12.

Abstract Von Willebrand factor A (VWA) domains are versatile protein interaction domains with N and C termini in close proximity placing spatial constraints on...   Read More

Reduced inflammatory threshold indicates skin barrier defect in transglutaminase 3 knockout mice

B2 WAGENER/ PAULSSONpublications Bognar P, Nemeth I, Mayer B, Haluszka D, Wikonkal N, Ostorhazi E, John S, Paulsson M, Smyth N, Pasztoi M, Buzas EI, Szipocs R, Kolonics A, Temesvari E, Karpati S; J Invest Dermatol. 2014 Jan;134(1):105-11. doi: 10.1038/jid.2013.307. Epub 2013 Jul 24.

Abstract   Recently, a transglutaminase 3 knockout (TGM3/KO) mouse was generated that showed impaired hair development, but no gross defects in the epidermal barrier,...   Read More

Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis

B2 WAGENER/ PAULSSONpublicationsSabatelli, P., Gualandi, F., Gara, S.K., Paulsson, M., Zamparelli, A., Martoni, E., Grumati, P., Pellegrini, C., Merlini, L., Ferlini, A. Maraldi, N.M., Bonaldo, P., Squarzoni, S. and Wagener, R., Matrix Biol. 2012 Apr;31(3):187-96. doi: 10.1016/j.matbio.2011.12.003. Epub 2011 Dec 30.

Abstract Collagen VI is a major extracellular matrix (ECM) protein with a critical role in maintaining skeletal muscle functional integrity. Mutations in COL6A1, COL6A2...   Read More

EMILIN- 3, a peculiar member of the EMILIN/Multimerin protein family, has a distinct expression pattern, forms oligomeric assemblies and serves as a pro TGF-

B2 WAGENER/ PAULSSONpublicationsSchiavinato, A., Becker, A.K., Zanetti, M., Corallo, D., Milanetto, M., Bizzotto, D., Bressan, G., Guljelmovic, M., Paulsson, M., Wagener, R., Braghetta, P. and Bonaldo, P., J Biol Chem. 2012 Mar 30;287(14):11498-515. doi: 10.1074/jbc.M111.303578. Epub 2012 Feb 10.

Abstract EMILIN-3 is a glycoprotein of the extracellular matrix belonging to a family that contains a characteristic N-terminal cysteine-rich EMI domain. Currently, EMILIN-3 is...   Read More

Enhanced deposition of cartilage oligomeric matrix protein is a common feature in fibrotic skin pathologies

B2 WAGENER/ PAULSSONpublicationsAgarwal P, Schulz JN, Blumbach K, Andreasson K, Heinegård D, Paulsson M, Mauch C, Eming SA, Eckes B, Krieg T; Matrix Biol. 2013 Aug 8;32(6):325-31. doi: 10.1016/j.matbio.2013.02.010. Epub 2013 Mar 15.

Abstract Skin fibrosis is characterized by activated fibroblasts and an altered architecture of the extracellular matrix. Excessive deposition of extracellular matrix proteins and altered...   Read More

PECAM1(+)/Sca1(+)/CD38(+) vascular cells transform into myofibroblast-like cells in skin wound repair

B2 WAGENER/ PAULSSONB4 ZIGRINO MAUCHpublications Etich J, Bergmeier V, Frie C, Kreft S, Bengestrate L, Eming S, Mauch C, Eckes B, Ulus H, Lund FE, Rappl G, Abken H, Paulsson M, Brachvogel B; PLoS One. 2013;8(1):e53262. doi: 10.1371/journal.pone.0053262. Epub 2013 Jan 4.

Abstract   Skin injury induces the formation of new blood vessels by activating the vasculature in order to restore tissue homeostasis. Vascular cells may...   Read More

Different domains in nidogen-1 and nidogen-2 drive basement membrane formation in skin organotypic cocultures

B11 BAUMANN/ZAUCKEB2 WAGENER/ PAULSSONpublicationsBechtel, M., Keller, M., Bloch, W., Sasaki, T., Boukamp, P., Zaucke, F., Paulsson, M., and Nischt, R., FASEB J. 2012 Sep;26(9):3637-48. doi: 10.1096/fj.11-194597. Epub 2012 May 23.

Abstract Nidogen-1 and nidogen-2 are homologous proteins found in all basement membranes (BMs). They show comparable binding activities in vitro and partially redundant functions...   Read More

Collagen XII and XIV, new partners of cartilage oligomeric matrix protein in the skin extracellular matrix suprastructure

B11 BAUMANN/ZAUCKEB2 WAGENER/ PAULSSONpublicationsAgarwal, P., Zwolanek, D., Keene, D. R., Schulz, J.N., Blumbach, K., Heinegard, D., Zaucke, F., Paulsson, M., Krieg, T., Koch, M., and Eckes, B., J Biol Chem. 2012 Jun 29;287(27):22549-59. doi: 10.1074/jbc.M111.335935. Epub 2012 May 9.

Abstract The tensile and scaffolding properties of skin rely on the complex extracellular matrix (ECM) that surrounds cells, vasculature, nerves, and adnexus structures and...   Read More

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