Dieter P. Reinhardt

dieter.reinhardt [at] (Email)
Tel:(514) 398-4243

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Research Interests

Extracellular matrix macromolecules are often large oligomers that polymerize into supra-structures at several hierarchic levels. They form insoluble fibrils or filaments that further assemble into tissue supramolecular structures. These supra-structures can be visualized as building blocks for formation and homeostasis of tissues and organs. On one hand, such supra-structures often confer structural support to tissues, and on the other hand they have numerous functional roles in development and homeostasis of tissues and organs. Deficiencies in formation and/or function of such supra-structures can lead to numerous genetic and degenerative disorders, affecting essentially all organs and tissues. One subset of these supramolecular structures is represented by microfibrillar systems, which are ubiquitously distributed in most elastic and non-elastic tissues. The fibrillin family of proteins forms the core of these supra-structures. Mutations in individual components of these systems give rise to a broad spectrum of genetic disorders generally termed microfibrillopathies including the Marfan syndrome (fibrillin-1), contractural arachnodactyly (fibrillin-2), and related disorders characterized by clinical symptoms in the skeletal, dental, cardiovascular, and the ocular systems. In order to advance our understanding of genotype-phenotype relationships in such disorders, information about microfibrillar systems in terms of biogenesis and functional aspects is urgently needed. The research in my group is driven by the "long term" hypothesis of the laboratory that microfibrillar systems are essential for biogenesis, homoeostasis, and degeneration processes of skeletal and cardiovascular tissues. Basically, my research projects address three important aspects of microfibril biology, which are (i) biogenesis of microfibrils, (ii) genotype-phenotype relationships of genetic disorders, and (iii) the ligand interaction repertoire and its functional significance. My laboratory uses a combined biochemical, cell biological, genetic, proteomic and recombinant protein technology approach to identify the components and mechanisms involved in these basic pathways of microfibril biology in health and disease. I expect that information obtained from this work will directly contribute to the design of diagnostic and therapeutic strategies for associated disorders.



Gilpin SE, Li Q, Evangelista-Leite D, Ren X, Reinhardt DP, Frey BL, Ott HC. (2017) Fibrillin-2 and Tenascin-C bridge the age gap in lung epithelial regeneration. Biomaterials 140: 212-219.

Hubmacher D, Schneider M, Berardinelli SJ, Takeuchi H, Willard B, Reinhardt DP, Haltiwanger RS, Apte SS. (2017) Unusual life cycle and impact on microfibril assembly of ADAMTS17, a secreted metalloprotease mutated in genetic eye disease. Sci Rep 7: 41871.

Kumra H, Reinhardt DP. (2017) Fibrillins. In Methods in Extra Cellular Matrix Biology (Mecham RP ed.), Elsevier. in press

Lee CS, Fu H, Baratang N, Rousseau J, Kumra H, Sutton VR, Niceta M, Ciolfi A, Yamamoto G, Bertola D, Marcelis CL, Lugtenberg D, Bartuli  A, Kim C, Hoover-Fong J, Sobreira N, Pauli R, Bacino C, Krakow D, Kariminejad A, Alvarez MA, Lausch E, Unger S, Superti-Furga A, Lu JT, Baylor-Hopkins Center for Mendelian Genomics, Cohn DH, Tartaglia M, Lee BH, **Reinhardt DP, **Campeau PM, **Co-Senior Authors. (2017) Mutations in fibronectin cause a subtype of spondylometaphyseal dysplasia with “corner fractures”. Am J Hum Genet: in press

Zeyer KA, Reinhardt DP. (2017) Fibrillin-1 in connective tissues. In Marfan Syndrome (Marfan Hilfe e. V. ed.), Springer, Berlin (Germany). pp 64-66.

Jondeau G, Ropers J, Regalado E, Braverman A, Evangelista A, Teixedo G, De Backer J, Muino-Mosquera L, Naudion S, Zordan C, Morisaki H, Morisaki Y, von Kodolitsch Y, Dupuis-Girod S, Morris SA, Jeremy R, Odent S, Ades LC, Bakshi M, Holman K, LeMaire S, Milleron O, Langeois M, Spentchian M, Aubart M, Boileau C, Pyeritz R, Milewicz DM, Montalcino Aortic Consortium**, **Reinhardt DP is member of this consortium. (2016) International Registry of Patients Carrying TGFBR1 or TGFBR2 Mutations: Results of the Montalcino Aortic Consortium. Circ Cardiovasc Genet 9: 548-558.

Kahle B, Schmidtke C, Hunzelmann N, Bartels C, Sievers HH, Reinhardt DP, Brinckmann J. (2016) The extracellular matrix signature in vein graft disease. Can J Cardiol 32: 1008.e1011-1007.

Kumra H, Reinhardt DP. (2016) Fibronectin-targeted drug delivery in cancer. Adv Drug Deliv Rev 97: 101-110.

Li CD, Hancock MA, Seghal P, Zhou S, Reinhardt DP, Philip A. (2016) Soluble CD109 binds TGF-ß and antagonizes TGF-ß signaling and responses. Biochem J 473: 537-547.

Loy C, Meghezi S, Lévesque L, Pezzoli D, Kumra H, Reinhardt DP, Kizhakkedathu JN, Mantovani D. (2016) A planar model of the vessel wall from cellularized collagen scaffolds: focus on cell–matrix interactions in mono-, bi- and tri-culture models. Biomater Sci 5: 153-162.

Schaefer L, Reinhardt DP. (2016) Extracellular matrix: Therapeutic tools and targets in cancer treatment. Adv Drug Deliv Rev 97: 1-3.

Guo DC, Gong L, Regalado ES, Santos-Cortez RL, Zhao R, Cai B, Veeraraghavan S, Prakash SK, Johnson RJ, Muilenburg A, Willing M, Jondeau G, Boileau C, Pannu H, Moran R, Debacker J, GenTac Investigators NHLBIGESP, Montalcino Aortic Consortium**, Bamshad MJ, Shendure J, Nickerson DA, Leal SM, Raman CS, Swindell EC, Milewicz DM, **Reinhardt DP is member of this consortium. (2015) MAT2A mutations predispose individuals to thoracic aortic aneurysms. Am J Hum Genet 96: 170-177.

Hinderer S, Shena N, Ringuette LJ, Hansmann J, Reinhardt DP, Brucker SY, Davis EC, Schenke-Layland K. (2015) In vitro elastogenesis - Instructing human vascular smooth muscle cells to generate an elastic fiber-containing extracellular matrix scaffold. Biomed Mater 10: 034102.

Hubmacher D, Wang LW, Mecham RP, Reinhardt DP, Apte SS. (2015) Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia: A novel mouse model providing insights on geleophysic dysplasia. Dis Model Mech 8: 487-499.

Kaur J, Reinhardt DP. (2015) Extracellular Matrix Molecules. In Stem Cell Biology and Tissue Engineering in Dental Science (Vishwakarma A, Sharpe PT, Shi S, Wang X, Ramalingam M eds.), Elsevier, New York. pp 25-43.

Regalado ES, Guo DC, Prakash S, Bensend TA, Flynn K, Estrera A, Safi H, Liang D, Hyland J, Child A, Arno G, Boileau C, Jondeau G, Braverman A, Moran R, Morisaki T, Morisaki H, Montalcino Aortic Consortium**, Pyeritz R, Coselli J, LeMaire S, Milewicz DM, **Reinhardt DP is member of this consortium. (2015) Aortic disease presentation and outcome associated with ACTA2 mutations. Circ Cardiovasc Genet 8: 457-464.

Zeyer K, Reinhardt DP. (2015) Engineered mutations in fibrillin-1 leading to Marfan syndrome act at the protein and cellular levels. Mutat Res Rev 765: 7-18.

Zeyer KA, Reinhardt DP. (2015) Fibrillin-containing microfibrils are key signal relay stations for cell function. J Cell Commun Signal 9: 309-325.

Hubmacher D, Bergeron E, Fagotto-Kaufmann C, Sakai LY, Reinhardt DP. (2014) Early fibrillin-1 assembly monitored through a modifiable recombinant cell approach. Biomacromolecules 15: 1456-1468.

Hubmacher D, Reinhardt DP, Plesec T, Schenke-Layland K, Apte SS. (2014) Human eye development is characterized by coordinated expression of fibrillin isoforms. Invest Ophthalmol Vis Sci 55: 7934-7944.

Marek I, Volkert G, Hilgers KF, Bieritz B, Rascher W, Reinhardt DP, Hartner A. (2014) Fibrillin-1 and alpha8 integrin are co-expressed in the glomerulus and interact to convey firm adhesion of mesangial cells. Cell Adh Migr 8: 389-395.

Muiznieks LD, Cirulis JT, van der Horst A, Reinhardt DP, Wuite GJL, Pomès R, Keeley FW. (2014) Modulated growth, stability and interactions of liquid-like coacervate assemblies of elastin. Matrix Biol 36: 39-50.

Murphy EC, Mörgelin M, Reinhardt DP, Björck L, Frick IM. (2014) Finegoldia magna uses its cell surface and extracellular proteins, FAF and SufA, to colonise and pass into deeper fractions of human skin. Mol Microbiol 94: 403-417.

Reinhardt DP. (2014) Microfibril-associated disorders: fibrillinopathies. J Glaucoma 23: S34-S35.

Sabatier L, Djokic J, Hubmacher D, Dzafik D, Nelea V, Reinhardt DP. (2014) Heparin/heparan sulfate controls fibrillin-1, -2 and -3 self-interactions in microfibril assembly. FEBS Lett 588: 2890-2897.

Beene LC, Wang LW, Hubmacher D, Keene DR, Reinhardt DP, Annis DS, Mosher DF, Mecham RP, Traboulsi EI, Apte SS. (2013) Non-selective assembly of fibrillin-1 and fibrillin-2 in the rodent ocular zonule and in cultured cells: Implications for Marfan syndrome. Invest Ophthalmol Vis Sci 54: 8337-8344.

Djokic J, Fagotto-Kaufmann C, Bartels R, Nelea V, Reinhardt DP. (2013) Fibulin-3, -4, and -5 are highly susceptible to proteolysis, interact with cells and heparin, and form multimers. J Biol Chem 288: 22821-22835.

Hummitzsch K, Irving-Rodgers HF, Hatzirodos N, Bonner W, Sabatier L, Reinhardt DP, Sado Y, Ninomiya Y, Wilhelm D, Rodgers RJ. (2013) A new model of development of the mammalian ovary and follicles. PLoS One 8: e55578.

Sabatier L, Djokic J, Fagotto-Kaufmann C, Chen M, Annis DS, Mosher DF, Reinhardt DP. (2013) Complex contributions of fibronectin to initiation and maturation of microfibrils. Biochem J 456: 283-295.

Tiedemann K, Boraschi-Diaz I, Rajakumar I, Kaur J, Roughley P, **Reinhardt DP, **Komarova SV, **Co-Senior Authors. (2013) Fibrillin-1 directly regulates osteoclast formation and function by a dual mechanism. J Cell Sci 126: 4187-4194.

Votteler M, Carvaja Berrio DA, Horke A, Sabatier L, Reinhardt DP, Nsair A, Aikawa E, Schenke-Layland K. (2013) Elastogenesis at the onset of human cardiac valve development. Development 140: 2345-2353.

Bader HL, Wang LW, Ho J, Tran T, Holden P, Fitzgerald J, Atit RP, Reinhardt DP, Apte SS. (2012) A disintegrin-like and metalloprotease domain containing thrombospondin type 1 motif-like 5 (ADAMTSL5) is a novel fibrillin-1-, fibrillin-2-, and heparin-binding member of the ADAMTS superfamily containing a netrin-like module. Matrix Biol 31: 398-411.

Kaur J, Reinhardt DP. (2012) Immobilized metal affinity chromatography co-purifies TGF-beta1 with histidine-tagged recombinant extracellular proteins. PLoS One 7: e48629.

Lammers G, Roth G, Heck M, Zengerle R, Tjabringa GS, Versteeg EM, Hafmans T, Wismans R, Reinhardt DP, Verwiel ET, Verwiel E, Zeeuwen PL, Schalkwijk J, Brock R, Daamen WF, van Kuppevelt TH. (2012) Construction of a microstructured collagen membrane mimicking the papillary dermis architecture and guiding keratinocyte morphology and gene expression. Macromol Biosci 12: 675-691.

Piha-Gossack A, Sossin W, Reinhardt DP. (2012) The evolution of extracellular fibrillins and their functional domains. PLoS One 7: e33560.

Hatzirodos N, Bayne RA, Irving-Rodgers HF, Hummitzsch K, Sabatier L, Lee S, Bonner W, Gibson MA, Rainey WE, Carr BR, Mason HD, Reinhardt DP, Anderson RA, Rodgers R. (2011) Linkage of regulators of TGF-beta activity in the fetal ovary to polycystic ovary syndrome. FASEB J 25: 2256-2265.

Hubmacher D, Reinhardt DP. (2011) Microfibrils and fibrillin. In Biology of Extracellular Matrix (Mecham RP ed.), Springer, Berlin. pp 233-265.

Hubmacher D, Sabatier L, Annis DS, Mosher DF, Reinhardt DP. (2011) Homocysteine modifies structural and functional properties of fibronectin and interferes with the fibronectin-fibrillin-1 interaction. Biochemistry 50: 5322-5332.

Kirschner R, Hubmacher D, Iyengar G, Kaur J, Fagotto-Kaufmann C, Bromme D, Bartels R, Reinhardt DP. (2011) Classical and neonatal Marfan syndrome mutations in fibrillin-1 cause differential protease susceptibilities and protein function. J Biol Chem 286: 32810-32823.

Sabatier L, Miosge N, Hubmacher D, Lin G, Davis EC, Reinhardt DP. (2011) Fibrillin-3 expression in human development. Matrix Biol 30: 43-52.

Brinckmann J, Hunzelmann N, Kahle B, Rohwedel J, Kramer J, Hubmacher D, Reinhardt DP. (2010) Enhanced fibrillin-2 expression is a general feature of wound healing and sclerosis: Potential alteration of cell attachment and storage of TGF-beta. Lab Invest 90: 739-752.

Hubmacher D, Cirulis JT, Miao M, Keeley FW, Reinhardt DP. (2010) Functional consequences of homocysteinylation of the elastic fiber proteins fibrillin-1 and tropoelastin. J Biol Chem 285: 1188-1198.

Schenke-Layland K, Xie J, Magnusson M, Angelis E, Li X, Wu K, Reinhardt DP, MacLellan WR, Hamm-Alvarez SF. (2010) Lymphocytic infiltration leads to degradation of lacrimal gland extracellular matrix structures in NOD mice exhibiting a Sjögren's syndrome-like exocrinopathy. Exp Eye Res 90: 223-237.

Hubmacher D, Reinhardt DP. (2009) One more piece in the fibrillin puzzle. Structure 7: 635-636.

Koenders MMJF, Yang L, Wismans RG, van der Werf KO, Reinhardt DP, Daamen W, Bennink ML, Dijkstra PJ, van Kuppevelt TH, Feijen J. (2009) Microscale mechanical properties of single elastic fibers: The role of fibrillin-microfibrils. Biomaterials 30: 2425-2432.

Sabatier L, Chen D, Fagotto-Kaufmann C, Hubmacher D, McKee MD, Annis DS, Mosher DF, Reinhardt DP. (2009) Fibrillin assembly requires fibronectin. Mol Biol Cell 20: 846-858.

Sabatier L, Chen D, Fagotto-Kaufmann C, Hubmacher D, McKee MD, Annis DS, Mosher DF, Reinhardt DP. (2009) Fibrillin assembly requires fibronectin - Incytes Highlight. Mol Biol Cell 20: 1119-1119.

Cirulis JT, Bellingham CM, Davis EC, Hubmacher D, Reinhardt DP, Mecham RP, Keeley FW. (2008) Fibrillins, fibulins and MAGP modulate the kinetics and morphology of in vitro self-assembly of a recombinant elastin-like polypeptide. Biochemistry 47: 12601-12613.

Daamen WF, Nillesen STM, Wismans R, Reinhardt DP, Hafmans T, Veerkamp JH, van Kuppevelt T. (2008) A biomaterial composed of collagen and solubilised elastin enhances angiogenesis and elastic fibre formation without calcification. Tissue Eng 14: 349-360.

Fricke M, Langer C, Brunner E, Sakai LY, Füzesi L, Reinhardt DP, Quondamatteo F. (2008) Fibrillin-1 in incisional hernias. An immunohistochemical study in scar and non-scar regions of human skin and muscle fasciae. J Anat 212: 674-685.

Hubmacher D, El-Hallous EI, Nelea V, Kaartinen MT, Lee ER, Reinhardt DP. (2008) Biogenesis of extracellular microfibrils-Multimerization of the fibrillin-1 C-terminus into bead-like structures enables self-assembly. Proc Natl Acad Sci U S A 105: 6548-6553.

Kloepper JE, Tiede S, Brinckmann J, Reinhardt DP, Fässler R, Paus R. (2008) Immunophenotyping of the human bulge region: The quest to define useful in situ markers for human epithelial hair follicle stem cells and their niche. Exp Dermatol 17: 592-609.

Wilkinson GA, Schittny JC, Reinhardt DP, Klein R. (2008) Role for ephrinB2 in postnatal lung alveolar development and elastic matrix integrity. Dev Dyn 237: 2220-2234.

El-Hallous EI, Sasaki T, Hubmacher D, Getie M, Tiedemann K, Brinckmann J, Bätge B, Davis EC, Reinhardt DP. (2007) Fibrillin-1 interactions with fibulins depend on the first hybrid domain and provide an adapter function to tropoelastin. J Biol Chem 282: 8935-8946.

Gnainsky Y, Kushnirsky Z, Bilu G, Hagai Y, Genina O, Volpin H, Bruck R, Spira G, Nagler A, Kawada N, Yoshizato K, Reinhardt DP, Libermann TA, Pines M. (2007) Gene expression during chemically induced liver fibrosis - effect of halofuginone on TGF-beta signaling. Cell Tissue Res 328: 153-166.

Schaefer L, Tsalastra W, Babelova A, Baliova M, Minnerup J, Sorokin L, Gröne HJ, Reinhardt DP, Pfeilschifter J, Iozzo RV, Schaefer RM. (2007) Decorin-mediated regulation of fibrillin-1 in the kidney involves the IGF-I receptor and mTOR. Am J Pathol 170: 301-315.

Drumea-Mirancea M, Wessels JT, Müller CA, Essl M, Eble JA, Tolosa M, Koch M, Reinhardt DP, Sixt M, Sorokin L, Stierhof YD, Schwarz H, Klein G. (2006) Characterization of a laminin-5 containing conduit system in the human thymus - a transport system for small molecules. J Cell Sci 119: 1396-1405.

Hartner A, Schaefer L, Porst M, Cordasic N, Gabriel A, Klanke B, Reinhardt DP, Hilgers KF. (2006) Role of fibrillin-1 in hypertensive and diabetic glomerular disease. Am J Physiol Renal Physiol 290: F1329-F1336.

Hubmacher D, Tiedemann K, Reinhardt DP. (2006) Fibrillins: From biogenesis of microfibrils to signaling functions. Curr Top Dev Biol 75: 93-123.

Porst M, Daniel C, Plank C, Schocklmann HO, Reinhardt DP, Hartner A. (2006) Induction and coexpression of latent transforming growth factor beta-binding protein 1 (LTBP-1) and fibrillin-1 in experimental glomerulonephritis. Nephron Exp Nephrol 102: e99-e104.

Porst M, Plank C, Bieritz B, Konik E, Fees H, Dötsch J, Hilgers KF, Reinhardt DP, Hartner A. (2006) Fibrillin-1 regulates mesangial cell attachment, spreading, migration and proliferation. Kidney Int 69: 450-456.

Reinhardt DP. (2006) Connective Tissue. In Marfan-Syndrom - Ein Ratgeber für Patienten, Angehörige und Betreuende (Marfan Hilfe e. V. ed.), Steinkopff Verlag, Darmstadt (Germany). pp 26-33.

Robinson P, Arteaga-Solis E, Baldock C, Collod-Beroud G, Booms P, De Paepe A, Dietz HC, Guo G, Handford PA, Judge DP, Kielty CM, Loeys B, Milewicz DM, Ney A, Ramirez F, Reinhardt DP, Tiedemann K, Whiteman P, Godfrey M. (2006) The molecular genetics of Marfan syndrome and related disorders. J Med Genet 43: 769-787.

Robinson PN, Reinhardt DP. (2006) Research Outlook. In Marfan-Syndrom - Ein Ratgeber für Patienten, Angehörige und Betreuende (Marfan Hilfe e. V. ed.), Steinkopff Verlag, Darmstadt (Germany). pp 143-148.

Wu J, Reinhardt DP, Batmunkh C, Lindenmaier W, Kretschmer-Kazemi Far R, Notbohm H, Hunzelmann N, Brinckmann J. (2006) Functional diversity of lysyl hydroxylase 2 in collagen synthesis of human dermal fibroblasts. Exp Cell Res 312: 3485-3494.

Booms P, Pregla R, Ney A, Barthel F, Reinhardt DP, Pletschacher A, Mundlos S, Robinson PN. (2005) RGD-containing fibrillin-1 fragments upregulate matrix metalloproteinase expression in cell culture: A potential factor in the pathogenesis of the Marfan syndrome. Hum Genet 116: 51-61.

Bouzeghrane F, Reinhardt DP, Reudelhuber T, Thibault G. (2005) Enhanced expression of fibrillin-1, a constituent of the myocardial extracellular matrix, in fibrosis. Am J Physiol Heart Circ Physiol 289: H982-H991.

Brinckmann J, Hunzelmann N, El-Hallous EI, Krieg T, Sakai LY, Krengel S, Reinhardt DP. (2005) Absence of autoantibodies against correctly folded recombinant fibrillin-1 protein in systemic scleroderma patients. Arthritis Res Ther 7: R1221-R1226.

Brinckmann J, Kim S, Wu J, Reinhardt DP, Batmunkh C, Metzen E, Notbohm H, Bank RA, Krieg T, Hunzelmann N. (2005) Interleukin 4 and prolonged hypoxia induce a higher gene expression of lysyl hydroxylase 2 and an altered cross-link pattern: Important pathogenetic steps in early and late stage of systemic scleroderma? Matrix Biol 24: 459-468.

Hubmacher D, Tiedemann K, Bartels R, Brinckmann J, Vollbrandt T, Bätge B, Notbohm H, Reinhardt DP. (2005) Modification of the structure and function of fibrillin-1 by homocysteine suggests a potential pathogenetic mechanism in homocystinuria. J Biol Chem 280: 34946-34955.

Sixt M, Kanazawa N, Selg M, Samson T, Roos G, Reinhardt DP, Pabst R, Lutz MB, Sorokin L. (2005) The conduit system transports soluble antigens from the afferent lymph to resident dendritic cells in the T cell area of the lymph node. Immunity 22: 19-29.

Tiedemann K, Sasaki T, Gustafsson E, Göhring W, Bätge B, Notbohm H, Timpl R, Wedel T, Schlötzer-Schrehardt U, Reinhardt DP. (2005) Microfibrils at basement membrane zones interact with perlecan via fibrillin-1. J Biol Chem 280: 11404-11412.

Gaber Y, Tiedemann K, Reinhardt DP, Brinckmann J. (2004) Changes in crosslinks in the collagenous connective tissue of lipodermatosclerosis. Phlebologie 33: 8-11.

Hartner A, Eifert T, Haas CS, Tuysuz C, Hilgers KF, Reinhardt DP, Amann K. (2004) Characterization of the renal phenotype in a mouse model of Marfan Syndrome. Virchows Arch 445: 382-388.

Kassner A, Tiedemann K, Notbohm H, Ludwig T, Mörgelin M, Reinhardt DP, Chu ML, Bruckner P, Grässel S. (2004) Molecular structure and interaction of recombinant human type XVI collagen. J Mol Biol 339: 835-853.

Lorena D, Darby IA, Reinhardt DP, Sapin V, Rosenbaum J, Desmouliere A. (2004) Fibrillin-1 expression in normal and fibrotic rat liver and in cultured hepatic fibroblastic cells: Modulation by mechanical stress and role in cell adhesion. Lab Invest 84: 203-212.

Schaefer L, Mihalik D, Babelova A, Krzyzankova M, Grone HJ, Iozzo RV, Young MF, Seidler DG, Lin G, Reinhardt DP, Schaefer RM. (2004) Regulation of fibrillin-1 by biglycan and decorin is important for tissue preservation in the kidney during pressure-induced injury. Am J Pathol 165: 383-396.

Tiedemann K, Bätge B, Reinhardt DP. (2004) Assembly of microfibrils. In Marfan Syndrome: A Primer for Clinicians and Scientists (Robinson PN, Godfrey M eds.), Landes Bioscience, Georgetown, Texas, USA. pp 130-142.

Vollbrandt T, Tiedemann K, El-Hallous EI, Lin G, Brinckmann J, John H, Bätge B, Notbohm H, Reinhardt DP. (2004) Consequences of cysteine mutations in calcium-binding epidermal growth factor modules of fibrillin-1. J Biol Chem 279: 32924-32931.

Charbonneau NL, Dzamba BJ, Ono RN, Keene DR, Corson GM, Reinhardt DP, Sakai LY. (2003) Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences. J Biol Chem 278: 2740-2749.

Eisenbeiss C, Martinez A, Hagedorn-Greiwe M, Reinhardt DP, Bätge B, Brinckmann J. (2003) Reduced skin thickness - a "new" minor symptom of the classical and hypermobile type of Ehlers-Danlos syndrome. Br J Dermatol 149: 850-852.

Isogai Z, Ono RN, Ushiro S, Keene DR, Chen Y, Mazzieri R, Charbonneau NL, Reinhardt DP, Rifkin DB, Sakai LY. (2003) Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein. J Biol Chem 278: 2750-2757.

Kassner A, Hansen U, Miosge N, Reinhardt DP, Aigner T, Bruckner-Tuderman L, Bruckner P, Grässel S. (2003) Discrete integration of collagen XVI into tissue-specific collagen fibrils or beaded microfibrils. Matrix Biol 22: 131-143.

Tiedemann K, Bätge B, Reinhardt DP. (2003) Marfan-Syndrom: Eine genetische Erkrankung des mikrofibrillären Systems. Bioforum 7-8: 456-457.

Isogai Z, Aspberg A, Keene DR, Ono RN, Reinhardt DP, Sakai LY. (2002) Versican interacts with fibrillin-1 and links extracellular microfibrils to other connective tissue networks. J Biol Chem 277: 4565-4572.

Lin G, Tiedemann K, Vollbrandt T, Peters H, Bätge B, Brinckmann J, Reinhardt DP. (2002) Homo- and heterotypic fibrillin-1 and -2 interactions constitute the basis for the assembly of microfibrils. J Biol Chem 277: 50795-50804.

Piecha D, Wiberg C, Mörgelin M, Reinhardt DP, Deak F, Maurer P, Paulsson M. (2002) Matrilin-2 interacts with itself and with other extracellular matrix proteins. Biochem J 367: 715-721.

Quondamatteo F, Reinhardt DP, Charbonneau NL, Pophal G, Sakai LY, Herken R. (2002) Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. Matrix Biol 21: 637-646.

Areida SK, Reinhardt DP, Müller PK, Fietzek PP, Köwitz J, Marinkovich MP, Notbohm H. (2001) Properties of the collagen type XVII ectodomain. Evidence for N- to C-terminal triple helix folding. J Biol Chem 276: 1594-1601.

Jensen SA, Reinhardt DP, Gibson MA, Weiss AS. (2001) MAGP-1, Protein interaction studies with tropoelastin and fibrillin-1. J Biol Chem 276: 39661-39666.

Tiedemann K, Bätge B, Müller PK, Reinhardt DP. (2001) Interactions of fibrillin-1 with heparin/heparan sulfate: Implications for microfibrillar assembly. J Biol Chem 276: 36035-36042.

Winkler J, Wirbelauer C, Lünsdorf H, Reinhardt DP, Laqua H. (2001) Immunohistochemical and charge-specific localization of anionic components of aggregates on the central anterior lens capsules from individuals with Pseudoexfoliation syndrome. Graefes Arch Clin Exp Ophthalmol 239: 952-960.

Handford PA, Downing AK, Reinhardt DP, Sakai LY. (2000) Fibrillin: from domain structure to supramolecular assembly. Matrix Biol 19: 457-470.

Reinhardt DP, Gambee JE, Ono RN, Bächinger HP, Sakai LY. (2000) Initial steps in assembly of microfibrils. Formation of disulfide-cross-linked multimers containing fibrillin-1. J Biol Chem 275: 2205-2210.

Reinhardt DP, Ono RN, Notbohm H, Müller PK, Bächinger HP, Sakai LY. (2000) Mutations in calcium-binding epidermal growth factor modules render fibrillin-1 susceptible to proteolysis: A potential disease-causing mechanism in Marfan syndrome. J Biol Chem 275: 12339-12345.

Wirbelauer C, Reinhardt DP, Lünsdorf H, Weber E, Laqua H, Winkler J. (2000) Charakterisierung der extrazellulären Matrix beim Pseudoexfoliationssyndrom. Focus MUL 17: 82-87.

Lönnqvist L, Reinhardt DP, Sakai LY, Peltonen L. (1998) Evidence for furin-type activity-mediated C-terminal processing of profibrillin-1 and interference in the processing by certain mutations. Hum Mol Genet 7: 2039-2044.

Keene DR, Jordan CD, Reinhardt DP, Ridgway CC, Ono RN, Corson GM, Fairhurst M, Sussman MD, Memoli VA, Sakai LY. (1997) Fibrillin-1 in human cartilage: developmental expression and formation of special banded fibers. J Histochem Cytochem 45: 1069-1082.

Pereira L, Andrikopoulos K, Tian J, Lee SY, Keene DR, Ono RN, Reinhardt DP, Sakai LY, Jensen-Biery N, Bunton T, Dietz HC, Ramirez F. (1997) Targeting of fibrillin-1 recapitulates the vascular phenotype of Marfan syndrome in the mouse. Nat Genet 17: 218-222.

Reinhardt DP, Mechling DE, Boswell BA, Keene DR, Sakai LY, Bächinger HP. (1997) Calcium determines the shape of fibrillin. J Biol Chem 272: 7368-7373.

Reinhardt DP, Ono RN, Sakai LY. (1997) Calcium stabilizes fibrillin-1 against proteolytic degradation. J Biol Chem 272: 1231-1236.

Pfaff M, Reinhardt DP, Sakai LY, Timpl R. (1996) Cell adhesion and integrin binding to recombinant human fibrillin-1. FEBS Lett 384: 247-250.

Reinhardt DP, Keene DR, Corson GM, Pöschl E, Bächinger HP, Gambee JE, Sakai LY. (1996) Fibrillin 1: organization in microfibrils and structural properties. J Mol Biol 258: 104-116.

Reinhardt DP, Sasaki T, Dzamba BJ, Keene DR, Chu ML, Göhring W, Timpl R, Sakai LY. (1996) Fibrillin-1 and fibulin-2 interact and are colocalized in some tissues. J Biol Chem 271: 19489-19496.

Mayer U, Zimmermann K, Mann K, Reinhardt DP, Timpl R, Nischt R. (1995) Binding properties and protease stability of recombinant human nidogen. Eur J Biochem 227: 681-686.

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