B2 WAGENER/ PAULSSON

Defective collagen VI α6 chain expression in the skeletal muscle of patients with collagen VI-related myopathies

B2 WAGENER/ PAULSSONTagliavini F, Pellegrini C, Sardone F, Squarzoni S, Paulsson M, Wagener R, Gualandi F, Trabanelli C, Ferlini A, Merlini L, Santi S, Maraldi NM, Faldini C, Sabatelli P; Biochim Biophys Acta. 2014 Sep;1842(9):1604-12. doi: 10.1016/j.bbadis.2014.05.033. Epub 2014 Jun 5.

Abstract Collagen VI is a non-fibrillar collagen present in the extracellular matrix (ECM) as a complex polymer; the mainly expressed form is composed of...   Read More

Col6a1 null mice as a model to study skin phenotypes in patients with collagen VI related myopathies: expression of classical and novel collagen VI variants during wound healing

B2 WAGENER/ PAULSSONB7 EMINGLettmann S, Bloch W, Maaß T, Niehoff A, Schulz JN, Eckes B, Eming SA, Bonaldo P, Paulsson M, Wagener R; PLoS One. 2014 Aug 26;9(8):e105686. doi: 10.1371/journal.pone.0105686. eCollection 2014.

Abstract Patients suffering from collagen VI related myopathies caused by mutations in COL6A1, COL6A2 and COL6A3 often also display skin abnormalities, like formation of...   Read More

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

B12 SENGLEB2 WAGENER/ PAULSSONRichardson 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/ PAULSSON 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/ PAULSSONBecker 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/ PAULSSON 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/ PAULSSONSabatelli, 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/ PAULSSONSchiavinato, 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/ PAULSSONAgarwal 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 MAUCH 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

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