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Dieter P. Reinhardt, Professor
Ling Li, Lab Manager
Valentin Nelea, Research Associate
Heena Kumra, PhD Student
Rongmo Zhang, PhD Student
Muthu Lakshmi, PhD Student
Hana Hakami, PhD Student
Neha Dinesh, PhD Student

Dieter P. Reinhardt, PhD, Professor

"The microfibril/elastic fiber system in health and disease"

- Applications for PhD and postdoctoral projects are welcome -

Research images

    Ling Li, Lab Manager
    Valentin Nelea, Research Associate
    Heena Kumra, Postdoctoral Fellow
    Rongmo Zhang, PhD Student
    Muthu Lakshmi, PhD Student
    Hana Hakami, PhD Student
    Neha Dinesh, PhD Student

    (514) 398-4243 (phone D. Reinhardt)
    (514) 398-5231 (phone lab)
    (514) 398-5375 (fax lab)

Connective tissue disorders represent a huge economic, physically debilitating, and psychosocial burden of illness. Genetic and degenerative disorders contribute to this burden. To improve this situation, basic and applied research is required on extracellular matrices (ECM), the major component of connective tissues, and their functional relationship with cells. ECM macromolecules often specifically interact to assemble into larger functional units - building blocks for tissues and organs. Such suprastructures confer structural support and provide a plethora of instructional roles for cells.

The laboratory is interested in components and mechanisms involved in the biogenesis, function and pathological aspects of the extracellular microfibril/elastic fiber system. Fibrillins and fibulins are two important protein families that constitute the backbone or are associated with microfibrils and elastic fibers. Mutations in these components lead to a wide spectrum of heritable connective tissue disorders, including Marfan syndrome, Beal's syndrome, stiff skin syndrome, systemic sclerosis, acromelic dysplasias, familial ectopia lentis, thoracic aortic aneurysms, familial mitral valve prolapse and myxomatous valvular disease, familial tall stature, cutis laxa, retinal dystrophy, macular degeneration and others.

The multi-disciplinary research in my lab is embedded in this framework addressing mechanisms of biogenesis and function of the microfibril/elastic fiber system in normal and pathologic situations. It is expected that the information obtained from this research program will not only benefit our basic understanding of the structure and function of microfibrils and elastic fibers, but will also help to design new preventative, diagnostic and therapeutic strategies for the associated disorders.


Citation Metrics (October 2019):
Total citations: 5632
Average citations per article: 48.5
Average citations per year: 201
h-index: 42
Citation details

Zhang, R., Kumra, H. and Reinhardt, D. P. (2020). Quantification of extracellular matrix fiber systems related to ADAMTS proteins. Methods Mol. Biol. in press: [no abstract available]

Nelea, V. and Reinhardt, D. P. (2020). Biophysical techniques to analyze elastic tissue extracellular matrix proteins. Methods Mol. Biol. in press: [no abstract available]

*Kumra, H., *Nelea, V., Hakami, H., Pagliuzza, A., Djokic, J., Xu, J., Yanagisawa, H., Reinhardt, D. P. and *Co-first authors contributed equally to this work (2019). Fibulin-4 exerts a dual role in LTBP-4L mediated matrix assembly and function. Proc. Natl. Acad. Sci. U. S. A. 116: 20428-20437 [Abstract]

Zeyer, K. A., Kumra, H., Hassan, A. and Reinhardt, D. P. (2019). The fibrillin-1 RGD integrin binding site regulates gene expression and cell function through microRNAs. J. Mol. Biol. 431: 401-421 [Abstract]

Burger, J., van Vliet, N., van Heijningen, P., Kumra, H., Kremers, G.-J., Alves, M., van Cappellen, G., Yanagisawa, H., Reinhardt, D. P., Kanaar, R., van der Pluijm, I. and Essers, J. (2019). Fibulin-4 deficiency differentially affects cytoskeleton structure and dynamics as well as TGFβ signaling. Cell. Signal. 58: 65-78 [Abstract]

Costantini, A., Valta, H., Baratang, N. V., Yap, P., Bertola, D., Yamamoto, G., Kim, C. A., Chen, J., Wierenga, K., Fanning, E. A., Escobar, L., McWalter, K., McLaughlin, H., Willaert, R., Begtrup, A., Alm, J. J., Reinhardt, D. P., Mäkitie, O. and Campeau, P. M. (2019). Novel fibronectin mutations expand the phenotype of the FN1-subtype of spondylometaphyseal dysplasia with "corner fractures". Bone 121: 163-171 [Abstract]

Dinesh, N. E. H. and Reinhardt, D. P. (2019). Inflammation in thoracic aortic aneurysms. Herz 44: 138-146 [Abstract]

Kumra, H., Dinesh, N. E. H. and Reinhardt, D. P. (2019). Lessons from tracheal tube development for understanding congenital tracheal malformations. Eur. Respir. J. 53: 1900127 [Abstract]

Wang, L. W., Kutz, W. E., Mead, T. J., Beene, L. C., Singh, S., Jenkins, M. W., Reinhardt, D. P. and Apte, S. S. (2019). Adamts10 inactivation in mice leads to persistence of ocular microfibrils subsequent to reduced fibrillin-2 cleavage. Matrix Biol. 77: 117-128 [Abstract]

Yin, X., Wanga, S., Fellows, A. L., Barallobre-Barreiro, J., Lu, R., Davaapil, H., Franken, R., Fava, M., Baig, F., Skroblin, P., Xing, Q., Koolbergen, D. R., Groenink, M., Zwinderman, K., Balm, R., de Vries, C. J., Mulder, B. J., Viner, R., Reinhardt, D. P., Sinha, S., Jahangiri, M., de Waard, V. and Mayr, M. (2019). Glycoproteomic analysis of the aortic extracellular matrix in Marfan patients. Arterioscler. Thromb. Vasc. Biol. 39: 1859-1873 [Abstract]

Kumra, H., Sabatier, L., Hassan, A., Sakai, T., Mosher, D. F., Brinckmann, J., Reinhardt, D. P. (2018). Roles of fibronectin isoforms in neonatal vascular development and matrix integrity. PLOS Biol. 16: e2004812 [Abstract]

Pezzoli, D., Di Paolo, J., Kumra, H., Fois, G., Candiani, G., **Reinhardt, D. P., **Mantovani, D. **Co-corresponding authors contributed equally to this work (2018). Fibronectin promotes elastin deposition, elasticity and mechanical strength in cellularised collagen-based scaffolds. Biomaterials 180: 130-142 [Abstract]

Camasão, D., Pezzoli, D., Loy, C., Kumra, H., Levesque, L., Reinhardt, D. P., Candiani, G., Mantovani, D. (2018). Increasing cell seeding density improves elastin expression and mechanical properties in collagen gel-based scaffolds cellularized with smooth muscle cells. Biotechnol. J. 1700768  [Abstract]

Kumra, H., Reinhardt, D. P. (2018). Fibrillins. Methods Cell Biol. 143: 223-246 [Abstract]

Lee, K.-W. L., Gade, P. S., Dong, L., Zhang, Z., Mubin Aral, A., Jin, G., Ding, X., Stowell, C. E. T., Umer Nisar, M., Kim, K., Reinhardt, D. P., Solari, M. G., Gorantla, V. S., Robertson, A. M., Wang, Y. (2018). A biodegradable synthetic graft for small arteries matches the performance of autologous vein in rat carotid arteries. Biomaterials 181: 67-80 [Abstract]

Regalado, E. S., Mellor-Crummey, L., De Backer, J., Braverman, A. C., Ades, L., Benedict, S., Bradley, T. J., Brickner, M. E., Chatfield, K. C., Child, A., Feist, C., Holmes, K. W., Iannucci, G., Lorenz, B., Mark, P., Morisaki, T., Morisaki, H., Morris, S. A., Mitchell, A. L., Ostergaard, J. R., Richer, J., Sallee, D., Shalhub, S., Tekin, M., Montalcino Aortic Consortium**, Estrera, A., Musolino, P., Yetman, A., Pyeritz, R., Milewicz, D. M. **Reinhardt DP is member of this consortium (2018). Clinical history and management recommendations of the smooth muscle dysfunction syndrome due to ACTA2 arginine 179 alterations. Genet. Med. 20: 1206-1215 [Abstract]

Lee, C. S., Fu, H., Baratang, N., Rousseau, J., Kumra, H., Sutton, V. R., Niceta, M., Ciolfi, A., Yamamoto, G., Bertola, D., Marcelis, C. L., Lugtenberg, D., Bartuli , A., Kim, C., Hoover-Fong, J., Sobreira, N., Pauli, R., Bacino, C., Krakow, D., Parboosingh, J., Yap, P., Kariminejad, A., McDonald, M. T., Aracena, M. I., Lausch, E., Unger, S., Superti-Furga, A., Lu, J. T., Baylor-Hopkins Center for Mendelian Genomics, Cohn, D. H., Tartaglia, M., Lee, B. H., **Reinhardt, D. P., **Campeau, P. M. **Co-corresponding authors contributed equally to this work (2017). Mutations in fibronectin cause a subtype of spondylometaphyseal dysplasia with “corner fractures”. Am. J. Hum. Genet. 101: 815-823 [Abstract]

Zeyer, K. A., Reinhardt, D. P. (2017). Fibrillin-1 in connective tissues.  In: Marfan Syndrome. Eds. Marfan Hilfe e. V. Springer, Berlin (Germany). Chp. 13, 64-66 [no abstract available]

Hubmacher, D., Schneider, M., Berardinelli, S. J., Takeuchi, H., Willard, B., Reinhardt, D. P., Haltiwanger, R. S., Apte, S. S. (2017). Unusual life cycle and impact on microfibril assembly of ADAMTS17, a secreted metalloprotease mutated in genetic eye disease. Sci. Rep. 7: 41871 [Abstract]

Gilpin, S. E., Li, Q., Evangelista-Leite, D., Ren, X., Reinhardt, D. P., Frey, B. L., Ott, H. C. (2017). Fibrillin-2 and Tenascin-C bridge the age gap in lung epithelial regeneration. Biomaterials 140: 212-219 [Abstract]

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

Loy, C., Meghezi, S., Lévesque, L., Pezzoli, D., Kumra, H., Reinhardt, D. P., Kizhakkedathu, J. N., 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 [Abstract]

Li, C. D., Hancock, M. A., Seghal, P., Zhou, S., Reinhardt, D. P., Philip, A. (2016). Soluble CD109 binds TGF-ß and antagonizes TGF-ß signaling and responses. Biochem. J. 473: 537-547 [Abstract]

Kumra, H., Reinhardt, D. P. (2016). Fibronectin-targeted drug delivery in cancer. Adv. Drug Deliv. Rev. 97: 101-110 [Abstract]

Kahle, B., Schmidtke, C., Hunzelmann, N., Bartels, C., Sievers, H. H., Reinhardt, D. P. and Brinckmann, J. (2016). The extracellular matrix signature in vein graft disease. Can. J. Cardiol. 32: 1008.e11-7 [Abstract]

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, S. A., Jeremy, R., Odent, S., Ades, L. C., Bakshi, M., Holman, K., LeMaire, S., Milleron, O., Langeois, M., Spentchian, M., Aubart, M., Boileau, C., Pyeritz, R., Milewicz, D. M., Montalcino Aortic Consortium**. **Reinhardt D. P. 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 [Abstract]

Zeyer, K. A. and Reinhardt, D. P. (2015). Fibrillin-containing microfibrils are key signal relay stations for cell function. J. Cell Commun. Signal. 9: 309-325 [Abstract]

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

Regalado, E. S., Guo, D. C., Prakash, S., Bensend, T. A., 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, D. M. **Reinhardt D. P. is member of this consortium (2015). Aortic disease presentation and outcome associated with ACTA2 mutations. Circ. Cardiovasc. Genet. 8: 457-464 [Abstract]

Kaur, J. and Reinhardt, D. P. (2015). Extracellular Matrix Molecules.  In: Stem Cell Biology and Tissue Engineering in Dental Science. Eds. Vishwakarma, A., Sharpe, P. T., Shi, S., Wang, X. and Ramalingam, M. Elsevier, New York. Chp. 3, 25-43 [no abstract available]

Hubmacher, D., Wang, L. W., Mecham, R. P., Reinhardt, D. P. and Apte, S. S. (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 [Abstract]

Hinderer, S., Shena, N., Ringuette, L. J., Hansmann, J., Reinhardt, D. P., Brucker, S. Y., Davis, E. C. and 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 [Abstract]

Guo, D. C., Gong, L., Regalado, E. S., Santos-Cortez, R. L., Zhao, R., Cai, B., Veeraraghavan, S., Prakash, S. K., Johnson, R. J., Muilenburg, A., Willing, M., Jondeau, G., Boileau, C., Pannu, H., Moran, R., Debacker, J., GenTac Investigators National Heart Lung Blood Institute Go Exome Sequencing Project, Montalcino Aortic Consortium**, Bamshad, M. J., Shendure, J., Nickerson, D. A., Leal, S. M., Raman, C. S., Swindell, E. C., Milewicz, D. M. **Reinhardt D. P. is member of this consortium (2015). MAT2A mutations predispose individuals to thoracic aortic aneurysms. Am. J. Hum. Genet. 96: 170-177 [Abstract]

Murphy, E. C., Mörgelin, M., Reinhardt, D. P., Björck, L., and Frick, I.-M. (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. [Abstract]

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

Reinhardt, D. P. (2014). Microfibril-associated disorders: fibrillinopathies. J. Glaucoma 23: S34-S35. [Abstract]

Marek, I., Volkert, G., Hilgers, K. F., Bieritz, B., Rascher, W., Reinhardt, D. P. and 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 [Abstract]

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

Hubmacher, D., Bergeron, E., Fagotto-Kaufmann, C., Sakai, L. Y., and Reinhardt, D. P. (2014). Early fibrillin-1 assembly monitored through a modifiable recombinant cell approach. Biomacromolecules 15: 1456-1468. [Abstract]

Muiznieks, L. D., Cirulis, J. T., van der Horst, A., Reinhardt, D. P., Wuite, G. J. L., Pomès, R. and Keeley, F. W. (2014). Modulated growth, stability and interactions of liquid-like coacervate assemblies of elastin. Matrix Biol. 36: 39-50 [Abstract]

Djokic, J., Fagotto-Kaufmann, C., Bartels, R., Nelea, V. and Reinhardt, D. P. (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 [Abstract]

Hummitzsch, K., Irving-Rodgers, H. F., Hatzirodos, N., Bonner, W., Sabatier, L., Reinhardt, D. P., Sado, Y., Ninomiya, Y., Wilhelm, D., and Rodgers, R. J. (2013). A new model of development of the mammalian ovary and follicles. PLoS ONE 8: e55578- [Abstract]

Votteler, M., Carvaja Berrio, D. A., Horke, A., Sabatier, L., Reinhardt, D. P., Nsair, A., Aikawa, E., and Schenke-Layland, K. (2013). Elastogenesis occurs at the early onset of human cardiac valve development. Development 140: 2345-2353. [Abstract]

Tiedemann, K., Boraschi-Diaz, I., Rajakumar, I., Kaur, J., Roughley, P., **Reinhardt, D. P., and **Komarova, S. V. **Co-last authors contributed equally to this work (2013). Fibrillin-1 directly regulates osteoclast formation and function by a dual mechanism. J. Cell Sci. 126: 4187-4194. [Abstract]

Beene, L. C., Wang, L. W., Hubmacher, D., Keene, D. R., Reinhardt, D. P., Annis, D. S., Mosher, D. F., Mecham, R. P., Traboulsi, E. I. and Apte, S. S. (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 [Abstract]

Sabatier, L., Djokic, J., Fagotto-Kaufmann, C., Chen, M., Annis, D. S., Mosher, D. F., and Reinhardt, D. P. (2013). Complex contributions of fibronectin to initiation and maturation of microfibrils. Biochem. J. 456: 283-295. [Abstract]

Bader, H. L., Wang, L. W., Ho, J., Tran, T., Holden, P., Fitzgerald, J., Atit, R. P., Reinhardt, D. P., Apte, S. S. (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 [Abstract]

Kaur, J. and Reinhardt, D. P. (2012). Immobilized metal affinity chromatography co-purifies TGF-beta1 with histidine-tagged recombinant extracellular proteins. PLoS ONE 7: e48629- [Abstract]

Lammers, G., Roth, G., Heck, M., Tjabringa, G. S., Versteeg, E. M., Hafmans, T., Wismans, R., Reinhardt, D. P., Verwiel, E., Zeeuwen, P. L., Schalkwijk, J., Brock, R., Daamen, W. F., and van Kuppevelt, T. H. (2012). Construction of a microstructured collagen membrane mimicking the papillary dermis architecture and guiding keratinocyte morphology and gene expression. Macromol. Biosci. 12: 675-691. [Abstract]

Piha-Gossack, A., Sossin, W. S., and Reinhardt, D. P. (2012). The evolution of extracellular fibrillins and their functional domains. PLoS ONE 7: e33560- [Abstract]

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

Hubmacher, D., Sabatier, L., Annis, D. S., Mosher, D. F., and Reinhardt, D. P. (2011). Homocysteine modifies structural and functional properties of fibronectin and interferes with the fibronectin-fibrillin-1 interaction. Biochemistry 50: 5322-5332. [Abstract]

Hatzirodos, N., Bayne, R. A., Irving-Rodgers, H. F., Hummitzsch, K., Sabatier, L., Lee, S., Bonner, W., Gibson, M. A., Rainey, W. E., Carr, B. R., Mason, H. D., Reinhardt, D. P., Anderson, R. A., and Rodgers, R. (2011). Linkage of regulators of TGF-beta activity in the fetal ovary to polycystic ovary syndrome. FASEB J. 25: 2256-2265. [Abstract]

Sabatier, L., Miosge, N., Hubmacher, D., Lin, G., Davis, E. C., and Reinhardt, D. P. (2011). Fibrillin-3 expression in human development. Matrix Biol. 30: 43-52. [Abstract]

Hubmacher, D. and Reinhardt, D. P. (2011) Microfibrils and fibrillin. In: Biology of Extracellular Matrix. Mecham, R. P. Springer. Chapter 7, Page 233-265 [Abstract]

Brinckmann, J., Hunzelmann, N., Kahle, B., Rohwedel, J., Kramer, J., Hubmacher, D., and Reinhardt, D. P. (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. [Abstract]

Hubmacher, D., Cirulis, Judith T., Miao, Ming, Keeley, F. W., and Reinhardt, D. P. (2010). Functional consequences of homocysteinylation of the elastic fiber proteins fibrillin-1 and tropoelastin. J. Biol. Chem. 285: 1188-1198. [Abstract]

Schenke-Layland, Katja, Xie, Jiansong, Magnusson, Mattias, Angelis, Ekaterini, Li, Xiaodong, Wu, K., Reinhardt, D. P., MacLellan, W. Robb, and Hamm-Alvarez, Sarah F. (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. [Abstract]

Hubmacher, D. and Reinhardt, D. P. (2009). One more piece in the fibrillin puzzle. Structure 7: 635-636. [Abstract]

Koenders, M. M. J. F, Yang, L., Wismans, R. G., van der Werf, K. O., Reinhardt, D. P., Daamen, W., Bennink, M. L., Dijkstra, P. J., van Kuppevelt, T. H., and Feijen, J. (2009). Microscale mechanical properties of single elastic fibers: The role of fibrillin-microfibrils. Biomaterials 30: 2425-2432. [Abstract]

Sabatier, L., Chen, D., Fagotto-Kaufmann, C., Hubmacher, D., McKee, M. D., Annis, D. S., Mosher, D. F., and Reinhardt, D. P. (2009). Fibrillin assembly requires fibronectin. Mol. Biol. Cell 20: 846-858. [Abstract]

Sabatier, L., Chen, D., Fagotto-Kaufmann, C., Hubmacher, D., McKee, M. D., Annis, D. S., Mosher, D. F., and Reinhardt, D. P. (2009). Fibrillin assembly requires fibronectin - Incytes Highlight. Mol. Biol. Cell 20: 1119-1119. [no abstract available]

Cirulis, Judith T., Bellingham, Catherine M., Davis, E. C., Hubmacher, D., Reinhardt, D. P., Mecham, R. P., and Keeley, F. W. (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. [Abstract]

Daamen, W. F., Nillesen, S. T. M., Wismans, R., Reinhardt, D. P., Hafmans, T., Veerkamp, J. H., and 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. [Abstract]

Fricke, M., Langer, C., Brunner, E., Sakai, L. Y., Füzesi, L., Reinhardt, D. P., and 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. [Abstract]

Hubmacher, D., El-Hallous, E. I., Nelea, V., Kaartinen, M. T., Lee, E. R., and Reinhardt, D. P. (2008). Biogenesis of extracellular microfibrils-Multimerization of the fibrillin-1 C-terminus into bead-like structures enables self-assembly. Proc. Natl. Acad. Sci. USA 105: 6548-6553. [Abstract]

Kloepper, J. E., Tiede, S., Brinckmann, J., Reinhardt, D. P., Fässler, R., and 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. [Abstract]

Wilkinson, G. A., Schittny, J. C., Reinhardt, D. P., and Klein, R. (2008). A role for ephrinB2 in postnatal lung alveolar development and elastic matrix integrity. Dev. Dyn. 237: 2220-2234. [Abstract]

El-Hallous, E. I., Sasaki, T., Hubmacher, D., Getie, M., Tiedemann, K., Brinckmann, J., Bätge, B., Davis, E. C., and Reinhardt, D. P. (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. [Abstract]

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

Schaefer, L., Tsalastra, W., Babelova, A., Baliova, M., Minnerup, J., Sorokin, L., Gröne, H.-J., Reinhardt, D. P., Pfeilschifter, J., Iozzo, R. V., and Schaefer, R. M. (2007). Decorin-mediated regulation of fibrillin-1 in the kidney involves the IGF-I receptor and mTOR. Am. J. Pathol. 170: 301-315. [Abstract]

Drumea-Mirancea, M, Wessels, J. T., Müller, C. A., Essl, M., Eble, J. A., Tolosa, M., Koch, M., Reinhardt, D. P., Sixt, M., Sorokin, L., Stierhof, Y.-D., Schwarz, H., and 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. [Abstract]

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

Hubmacher, D., Tiedemann, K., and Reinhardt, D. P. (2006). Fibrillins: From Biogenesis of Microfibrils to Signaling Functions. Curr. Top. Dev. Biol. 75: 93-123. [Abstract]

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

Porst, M., Daniel, C., Plank, C., Schocklmann, H. O., Reinhardt, D. P., and 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. [Abstract]

Reinhardt, D. P. (2006) Connective Tissue (German). In: Marfan-Syndrom - Ein Ratgeber für Patienten, Angehörige und Betreuende. Marfan Hilfe (Deutschland) e.V. Steinkopff Verlag, Darmstadt (Germany). 26-33. [no abstract available]

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

Robinson, P. N. and Reinhardt, D. P. (2006) Research Outlook (German). In: Marfan-Syndrom - Ein Ratgeber für Patienten, Angehörige und Betreuende. Marfan Hilfe (Deutschland) e.V. Steinkopff Verlag, Darmstadt (Germany). 143-148. [no abstract available]

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

Booms, P., Pregla, R., Ney, A., Barthel, F., Reinhardt, D. P., Pletschacher, A., Mundlos, S., and Robinson, P. N. (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. [Abstract]

Bouzeghrane, F., Reinhardt, D. P., Reudelhuber, T., and 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. [Abstract]

Brinckmann, J., Hunzelmann, N., El-Hallous, E. I., Krieg, T., Sakai, L. Y., Krengel, S, and Reinhardt, D. P. (2005). Absence of autoantibodies against correctly folded recombinant fibrillin-1 protein in systemic scleroderma patients. Arthritis Res. Ther. 7: R1221-R1226. [Abstract]

Brinckmann, J., Kim, S., Wu, J., Reinhardt, D. P., Batmunkh, C., Metzen, E., Notbohm, H., Bank, R. A., Krieg, T., and 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. [Abstract]

Hubmacher, D., Tiedemann, K., Bartels, R., Brinckmann, J., Vollbrandt, T., Bätge, B., Notbohm, H., and Reinhardt, D. P. (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. [Abstract]

Sixt, M., Kanazawa, N., Selg, M., Samson, T., Roos, G., Reinhardt, D. P., Pabst, R., Lutz, M. B., and 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. [Abstract]

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

Gaber, Y, Tiedemann, K., Reinhardt, D. P., and Brinckmann, J. (2004). Changes in crosslinks in the collagenous connective tissue of lipodermatosclerosis. Phlebologie 33: 8-11. [no abstract available]

Hartner, A., Eifert, T., Haas, C. S., Tuysuz, C., Hilgers, K. F., Reinhardt, D. P., and Amann, K. (2004). Characterization of the renal phenotype in a mouse model of Marfan Syndrome. Virchows Arch. 445: 382-388. [Abstract]

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

Lorena, D., Darby, I. A., Reinhardt, D. P., Sapin, V., Rosenbaum, J., and 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. [Abstract]

Schaefer, L., Mihalik, D., Babelova, A., Krzyzankova, M., Grone, H. J., Iozzo, R. V., Young, M. F., Seidler, D. G., Lin, G., Reinhardt, D. P., and Schaefer, R. M. (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. [Abstract]

Tiedemann, K., Bätge, B., and Reinhardt, D. P. (2004) Assembly of microfibrils. In: Marfan Syndrome: A Primer for Clinicians and Scientists. Robinson, P. N. and Godfrey, M. Landes Bioscience, Georgetown, Texas, USA. 130-142. [no abstract available]

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

Charbonneau, N. L., Dzamba, B. J., Ono, R. N., Keene, D. R., Corson, G. M., Reinhardt, D. P., and Sakai, L. Y. (2003). Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences. J. Biol. Chem. 278: 2740-2749. [Abstract]

Eisenbeiss, C., Martinez, A., Hagedorn-Greiwe, M., Reinhardt, D. P., Bätge, B., and 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. [Abstract]

Isogai, Z., Ono, R. N., Ushiro, S., Keene, D. R., Chen, Y., Mazzieri, R., Charbonneau, N. L., Reinhardt, D. P., Rifkin, D. B., and Sakai, L. Y. (2003). Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-associated protein. J. Biol. Chem. 278: 2750-2757. [Abstract]

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

Tiedemann, K., Bätge, B., and Reinhardt, D. P. (2003). Marfan-Syndrom: Eine genetische Erkrankung des mikrofibrillären Systems. Bioforum 7-8: 456-457. [no abstract available]

Isogai, Z., Aspberg, A., Keene, D. R., Ono, R. N., Reinhardt, D. P., and Sakai, L. Y. (2002). Versican interacts with fibrillin-1 and links extracellular microfibrils to other connective tissue networks. J. Biol. Chem. 277: 4565-4572. [Abstract]

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

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

Quondamatteo, F., Reinhardt, D. P., Charbonneau, N. L., Pophal, G., Sakai, L. Y., and Herken, R. (2002). Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development. Matrix Biol. 21: 637-646. [Abstract]

Areida, S. K., Reinhardt, D. P., Müller, P. K., Fietzek, P. P., Köwitz, J., Marinkovich, M. P., and 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. [Abstract]

Jensen, S. A., Reinhardt, D. P., Gibson, M. A., and Weiss, A. S. (2001). MAGP-1, Protein interaction studies with tropoelastin and fibrillin-1. J. Biol. Chem. 276: 39661-39666. [Abstract]

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

Winkler, J., Wirbelauer, C., Lünsdorf, H., Reinhardt, D. P., and 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. [Abstract]

Handford, P. A., Downing, A. K., Reinhardt, D. P., and Sakai, L. Y. (2000). Fibrillin: from domain structure to supramolecular assembly. Matrix Biol. 19: 457-470. [Abstract]

Reinhardt, D. P., Gambee, J. E., Ono, R. N., Bächinger, H. P., and Sakai, L. Y. (2000). Initial steps in assembly of microfibrils. Formation of disulfide-cross-linked multimers containing fibrillin-1. J. Biol. Chem. 275: 2205-2210. [Abstract]

Reinhardt, D. P., Ono, R. N., Notbohm, H., Müller, P. K., Bächinger, H. P., and Sakai, L. Y. (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. [Abstract]

Wirbelauer, C., Reinhardt, D. P., Lünsdorf, H., Weber, E., Laqua, H., and Winkler, J. (2000). Charakterisierung der extrazellulären Matrix beim Pseudoexfoliationssyndrom. Focus MUL 17: 82-87. [no abstract available]

Lönnqvist, L., Reinhardt, D. P., Sakai, L. Y., and 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. [Abstract]

Keene, D. R., Jordan, C. D., Reinhardt, D. P., Ridgway, C. C., Ono, R. N., Corson, G. M., Fairhurst, M., Sussman, M. D., Memoli, V. A., and Sakai, L. Y. (1997). Fibrillin-1 in human cartilage: developmental expression and formation of special banded fibers. J. Histochem. Cytochem. 45: 1069-1082. [Abstract]

Pereira, L., Andrikopoulos, K., Tian, J., Lee, S. Y., Keene, D. R., Ono, R. N., Reinhardt, D. P., Sakai, L. Y., Jensen-Biery, N., Bunton, T., Dietz, H. C., and Ramirez, F. (1997). Targeting of fibrillin-1 recapitulates the vascular phenotype of Marfan syndrome in the mouse. Nat. Genet. 17: 218-222. [Abstract]

Reinhardt, D. P., Mechling, D. E., Boswell, B. A., Keene, D. R., Sakai, L. Y., and Bächinger, H. P. (1997). Calcium determines the shape of fibrillin. J. Biol. Chem. 272: 7368-7373. [Abstract]

Reinhardt, D. P., Ono, R. N., and Sakai, L. Y. (1997). Calcium stabilizes fibrillin-1 against proteolytic degradation. J. Biol. Chem. 272: 1231-1236. [Abstract]

Pfaff, M., Reinhardt, D. P., Sakai, L. Y., and Timpl, R. (1996). Cell adhesion and integrin binding to recombinant human fibrillin-1. FEBS Lett. 384: 247-250. [Abstract]

Reinhardt, D. P., Sasaki, T., Dzamba, B. J., Keene, D. R., Chu, M. L., Göhring, W., Timpl, R., and Sakai, L. Y. (1996). Fibrillin-1 and fibulin-2 interact and are colocalized in some tissues. J. Biol. Chem. 271: 19489-19496. [Abstract]

Reinhardt, D. P., Keene, D. R., Corson, G. M., Pöschl, E., Bächinger, H. P., Gambee, J. E., and Sakai, L. Y. (1996). Fibrillin 1: organization in microfibrils and structural properties. J. Mol. Biol. 258: 104-116. [Abstract]

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

Reinhardt, D. P., Chalberg, S. C., and Sakai, L. Y. (1995) The structure and function of fibrillin. In: The molecular biology and pathology of elastic tissues. John Wiley and Sons, Chichester, England. 128-147. [Abstract]

Aumailley, M., Battaglia, C., Mayer, U., Reinhardt, D. P., Nischt, R., Timpl, R., and Fox, J. W. (1993). Nidogen mediates the formation of ternary complexes of basement membrane components. Kidney Int. 43: 7-12. [Abstract]

Reinhardt, D. P., Mann, K., Nischt, R., Fox, J. W., Chu, M. L., Krieg, T., and Timpl, R. (1993). Mapping of nidogen binding sites for collagen type IV, heparan sulfate proteoglycan, and zinc. J. Biol. Chem. 268: 10881-10887. [Abstract]

Kübler, D., Reinhardt, D. P., Reed, J., Pyerin, W., and Kinzel, V. (1992). Atrial natriuretic peptide is phosphorylated by intact cells through cAMP-dependent ecto-protein kinase. Eur. J. Biochem. 206: 179-186. [Abstract]

Fox, J. W., Mayer, U., Nischt, R., Aumailley, M., Reinhardt, D. P., Wiedemann, H., Mann, K., Timpl, R., Krieg, T., Engel, J., and Chu, M. L. (1991). Recombinant nidogen consists of three globular domains and mediates binding of laminin to collagen type IV. EMBO J. 10: 3137-3146. [Abstract]