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Publications | Grants

Selected Publications

• Csorba K, Sesarman A, Oswald E, Feldrihan V, Fritsch A, Sitaru C. Cross-reactivity of autoantibodies from patients with epidermolysis bullosa acquisita with murine collagen VII. Cell Mol Life Sci
• Mahoney MY, Sadowski S, Brennan D, Pikander P, Saukko P, Wahl J, Aho H, Heikinheimo K, Bruckner-Tuderman L, Fertala A, Peltonen J, Uitto J, Peltonen S. Compound Heterozygous Desmoplakin Mutations Result in a Phenotype with a Combination of Myocardial, Skin, Hair, and Enamel Abnormalities. J Invest Dermatol.
• Natsuga K, Nishie W, Akiyama M, Nakamura H, Shinkuma S, McMillan JR, Nagasaki A, Has C, Ouchi T, Ishiko A, Hirako Y, Owaribe K, Sawamura D, Bruckner-Tuderman L, Shimizu H. Plectin expression patterns determine two distinct subtypes of epidermolysis bullosa simplex. Hum Mutat.
• Sitaru AG, Sesarman A, Mihai S, Chiriac MT, Zillikens D, Hultman P, Solbach W, Sitaru C. T Cells Are Required for the Production of Blister-Inducing Autoantibodies in Experimental Epidermolysis Bullosa Acquisita. J Immunol.
• Bornholdt D, Oeffner F, König A, Happle R, Alanay Y, Ascherman J, Benke PJ, Boente Mdel C, van der Burgt I, Chassaing N, Ellis I, Francisco CR, Della Giovanna P, Hamel B, Has C, Heinelt K, Janecke A, Kastrup W, Loeys B, Lohrisch I, Marcelis C, Mehraein Y, Nicolas ME, Pagliarini D, Paradisi M, Patrizi A, Piccione M, Piza-Katzer H, Prager B, Prescott K, Strien J, Utine GE, Zeller MS, Grzeschik KH. PORCN mutations in focal dermal hypoplasia: coping with lethality. Hum Mutat. 2009 May;30(5):618-28.
• Franzke CW, Bruckner-Tuderman L, Blobel CP. Shedding of collagen XVII/BP180 in skin depends on both ADAM10 and ADAM9. J Biol Chem. 2009 Aug 28;284(35):23386-96.
• Fritsch A, Spassov S, Elfert S, Schlosser A, Gache Y, Meneguzzi G, Bruckner-Tuderman L. Dominant negative effects of COL7A1 mutations can be rescued by controlled over-expression of normal collagen VII. J Biol Chem 2009 Oct 30;284(44):30248-56.
• Has C, Herz C, Zimina E, Qu HY, He Y, Zhang ZG, Wen TT, Gache Y, Aumailley M, Bruckner-Tuderman L. Kindlin-1 Is required for RhoGTPase-mediated lamellipodia formation in keratinocytes. Am J Pathol. 2009 Oct;175(4):1442-52.
• Hofmann SC, Voith U, Schönau V, Sorokin L, Bruckner-Tuderman L, Franzke CW. Plasmin Plays a Role in the In Vitro Generation of the Linear IgA Dermatosis Antigen LADB97. J Invest Dermatol. 2009 Jul;129(7):1730-9.
• Kern JS, Grüninger G, Imsak R, Müller ML, Schumann H, Kiritsi D, Emmert S, Borozdin W, Kohlhase J, Bruckner-Tuderman L, Has C. 42 novel COL7A1 mutations and the role of a frequent SNP in the MMP1 promoter in modulation of disease severity in a large European dystrophic Epidermolysis bullosa cohort. Br J Dermatol. 2009 Nov;161(5):1089-97.
• Kern JS, Loeckermann S, Fritsch A, Hausser I, Roth W, MaginTM, Mack C, Müller ML, Paul O, Ruther P, Bruckner-Tuderman L. Mechanisms of fibroblast cell therapy for dystrophic epidermolysis bullosa: high stability of collagen VII favors long-term skin integrity. Mol Ther. 2009 Sep;17(9):1605-15.
• Roth W, Reuter U, Wohlenberg C, Bruckner-Tuderman L, Magin TM. Cytokines as genetic modifiers in K5-/- mice and in human epidermolysis bullosa simplex. Hum Mutat. 2009 May;30(5):832-41.
• Fritsch A*, Loeckermann S*, Kern JS*, Braun A, Bösl MR, Bley T, Schumann H, Elverfeldt D, Paul D, Erlacher M, Berens von Rautenfeld D, Hausser I, Fässler R, Bruckner-Tuderman L. A hypomorphic mouse model for dystrophic epidermolysis bullosa reveals disease mechanisms and responds to fibroblast therapy. J Clin Invest. 2008 May; 118(5):1669-79 (*equal contribution)
• Villone D, Fritsch A, Koch M, Bruckner-Tuderman L, Hansen U, Bruckner P.Supramolecular interactions in the dermo-epidermal junction zone: Anchoring fibril-collagen VII tightly binds to banded collagen fibrils. J Biol Chem. 2008 Sep 5; 283(36):24506-13
• Zimina EP, Hofmann SC, Fritsch A, Kern JS, Sitaru C, Bruckner-Tuderman L. Bullous Pemphigoid Autoantibodies Preferentially Recognize Phosphoepitopes in Collagen XVII. J Invest Dermatol. 2008 Nov;128(11):2736-9
• Hofmann SC, Voith U, Sasaki T, Trueb RM, Nischt R, Bruckner-Tuderman L. The Autoantigen in Anti-p200 Pemphigoid Is Synthesized by Keratinocytes and Fibroblasts and Is Distinct from Nidogen-2. J Invest Dermatol. 2007 Oct; 127(10):2438-44.
• Kern JS, Herz C, Haan E, Moore D, Nottelmann S, von Lilien T, Greiner P, Schmitt-Graeff A, Opitz OG, Bruckner-Tuderman L, Has C. Chronic colitis due to an epithelial barrier defect: the role of kindlin-1 isoforms. J Pathol. 2007 Dec; 213(4):462-70.
• Zimina EP, Fritsch A, Schermer B, Bakulina AY, Bashkurov M, Benzing T, Bruckner-Tuderman L. Extracellular phosphorylation of collagen XVII by ecto-casein kinase 2 inhibits ectodomain shedding. J Biol Chem. 2007 Aug 3;282(31):22737-46. Epub 2007 Jun 1.
• Franzke CW, Has C, Schulte C, Huilaja L, Tasanen K, Aumailley M, Bruckner-Tuderman L. C-terminal truncation impairs glycosylation of transmembrane collagen XVII and leads to intracellular accumulation. J Biol Chem. 2006 Oct 6;281(40):30260-8.
• Herz C, Aumailley M, Schulte C, Schlotzer-Schrehardt U, Bruckner-Tuderman L, Has C. Kindlin-1 is a phosphoprotein involved in regulation of polarity, proliferation and motility of epidermal keratinocytes. J Biol Chem. 2006 Nov; 281(47):36082-90.
• Kern JS, Kohlhase J, Bruckner-Tuderman L, Has C. Expanding the COL7A1 mutation database: novel and recurrent mutations and unusual genotype-phenotype constellations in 41 patients with dystrophic epidermolysis bullosa. J Invest Dermatol. 2006 May;126(5):1006-12.
• Schacht V, Dadras SS, Johnson LA, Jackson DG, Hong YK, Detmar M. Up-regulation of the lymphatic marker podoplanin, a mucin-type transmembrane glycoprotein, in human squamous cell carcinomas and germ cell tumors. Am J Pathol. 2005 Mar;166(3):913-21.
• Zimina EP, Bruckner-Tuderman L, Franzke CW. Shedding of collagen XVII ectodomain depends on plasma membrane microenvironment. J Biol Chem. 2005 Oct 7;280(40):34019-24.
• Franzke CW, Tasanen K, Borradori L, Huotari V, Bruckner-Tuderman L. Shedding of collagen XVII/BP180: structural motifs influence cleavage from cell surface. J Biol Chem. 2004 Jun 4;279(23):24521-9.
• Baldeschi C, Gache Y, Rattenholl A, Bouille P, Danos O, Ortonne JP, Bruckner-Tuderman L, Meneguzzi G. Genetic correction of canine dystrophic epidermolysis bullosa mediated by retroviral vectors. Hum Mol Genet. 2003 Aug 1;12(15):1897-905.
• Schacht V, Ramirez MI, Hong YK, Hirakawa S, Feng D, Harvey N, Williams M, Dvorak AM, Dvorak HF, Oliver G, Detmar M. T1alpha/podoplanin deficiency disrupts normal lymphatic vasculature formation and causes lymphedema. EMBO J. 2003 Jul 15;22(14):3546-56.
• Franzke CW, Tasanen K, Schacke H, Zhou Z, Tryggvason K, Mauch C, Zigrino P, Sunnarborg S, Lee DC, Fahrenholz F, Bruckner-Tuderman L. Transmembrane collagen XVII, an epithelial adhesion protein, is shed from the cell surface by ADAMs. EMBO J. 2002 Oct 1;21(19):5026-35.

Reviews and Commentaries

• Hundorfean G, Neurath MF, Sitaru C. Autoimmunity against type VII collagen in inflammatory bowel disease. J Cell Mol Med. 2009 Oct 31. [Epub ahead of print] 
• Van Agtmael T, Bruckner-Tuderman L. Basement membranes and human disease. Cell Tissue Res. 2010; 339:167-88.
• Bruckner-Tuderman L. Dystrophic epidermolysis bullosa: pathogenesis and clinical features. Dermatol Clin. 2010;28:107-14.
• Has C. Kern JS. Collagen XVII. Dermatol Clin. 2010 Jan;28(1):61-6.
• Bruckner-Tuderman, L. Can type VII collagen injections cure dystrophic epidermolysis bullosa? Mol Ther. 2009;17:6-7.
• Has C. Kindler syndrome : A new bullous dermatosis. Hautarzt 2009 Aug;60(8):622-6.
• Sitaru C. Bullous pemphigoid: a prototypical antibody-mediated organ-specific autoimmune disease. J Invest Dermatol 2009 Apr;129(4):822-4.
• Bruckner-Tuderman L. Secondary modifiers and the phenotypic variability of junctional epidermolysis bullosa. Acta Derm Venereol. 2008;88(5):436.
• Bruckner-Tuderman L. European Dermatology Forum: skin diseases in Europe. Skin diseases with a high public health impact: Epidermolysis bullosa. Eur J Dermatol. 2008 Mar-Apr;18(2):214-6.
• Fine JD, Eady RA, Bauer EA, Bauer JW, Bruckner-Tuderman L, Heagerty A, Hintner H, Hovnanian A, Jonkman MF, Leigh I, McGrath JA, Mellerio JE, Murrell DF, Shimizu H, Uitto J, Vahlquist A, Woodley D, Zambruno G. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol. 2008 Jun;58(6):931-50.

Research Grant Support

German Research Council (DFG)

• Transmembrane collagene XVII:Role in cell migration and -invasion (SFB 850, project B6). Pl: Leena Bruckner-Tuderman, Claus-Werner Franzke
• Kindlin-1 and molecular mechanisms of the Kindler Syndrome (Ha 5663/1-1), Pl: Cristina Has
• Function of podoplanin in lymphangiogenesis (SFB TR-23, project A5). PI: Vivien Schacht
• Start-up project Excellence cluster BIOSS:Regulation of epithelial cell-cell adhesion: Signaling pathways instrumental in acantholysis (BIOSS-B13). Pl: Cassian Sitaru
• Inhibition of the protein therapy-induced immune response in dystrophic epidermolysis bullosa (SI 1281/2-1).Pl: Cassian Sitaru
• Molecular mechanisms of autoantibody-induced granulocyte-dependent epithelial damage (SI 1281/4-1). Pl: Cassian Sitaru

German Ministry for Education and Research (BMBF)

• Network of Excellence for Rare Diseases, coordinator: Leena Bruckner-Tuderman (www.netzwerk-eb.de). Project 9 “Genotype-phenotype correlations and gene therapy strategies for dystrophic epidermolysis bullosa”, PI: Leena Bruckner-Tuderman
• Center for Chronic Immunodediciency (CCI) , Tandemproject 10: Antimicrobial peptides in S. aureus colonization and infection, Pl: Leena Bruckner-Tuderman, Silke Hofmann
• Stem cell therapy for severe inherited skin fragelity disorders, coordinator: Martin Röcken, Tübingen. Project 3 "Stemm cell therapy for severe dystrophic epidermolysis bullosa", Pl: Leena Bruckner-Tuderman, Johannes Kern

Grants from the European Union (EU)

• "Pemphigus", FP7-HEALTH-2007-A, coordinators: M. Hertl, C. Veldmann, Marburg, Pl: Leena Bruckner-Tuderman

E-rare (ERA-Net for research programs on rare diseases)

• International Kindler Syndrome Network (Kindlernet), coordinator: Leena Bruckner-Tuderman, (www.kindlernet.org).Pl: Leena Bruckner-Tuderman

• Id entification of revertant mosaicism in epidermolysis bullosa and subsequently using the revertant keratinocytes in a pre-clinical mouse model suitable to test revertant cell therapy, coordinator: Marcel Jonkman, Groningen (NL), Pl: Leena Bruckner-Tuderman

 Grants from the University of Freiburg

• Role of ADAM17 in the biology and pathology in the skin (FRA725/09). Pl: Claus-Werner Franzke