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个人简介

Hans studied biochemistry at the Freie Universität Berlin, Germany, and moved to the field of human genetics for his PhD studies. He obtained a PhD for a project exploring the genetic basis of a group of monogenic skin diseases, which is a major research interest until today. He continued working on disorders of keratinisation with Prof. A. Reis (then Institute of Human Genetics, Charité Berlin) and Prof. I. Leigh (then Imperial Cancer Reasearch Fund, London) before moving to the Gene Mapping Center (director Prof. A. Reis) at the Max Delbrück Center for Molecular Medicine, Berlin. In 2005 he was appointed Junior Research Group leader at the University of Cologne and has been head of the Division of Dermatogenetics at the Cologne Center for Genomics (director Prof. P. Nürnberg), and he is affiliated with the University of Cologne until today, to oversee research projects and deliver lectures at the Faculty of Medicine. He completed the habilitation and was awarded the venia legendi in 2012. He has been principal investigator at the Center for Molecular Medicine Cologne and the Cologne Cluster of Excellence on Cellular Stress Responses in Aging-Associated Diseases and established several collaborative research initiatives. He is a board member of the Network for Ichthyoses and Related Keratinisation Disorders (NIRK) and coordinator of the Network on Rare Congenital Skin Diseases (SkinDev) funded by European Research Agencies. From 2013 he was involved in teaching, research and diagnostics of genetic skin diseases at the Institute of Human Genetics of the Medical University of Innsbruck, Austria, and joined the University of Huddersfield in 2015 as a Reader in Biological Sciences.

研究领域

Skin is the largest human organ. It represents the interface of the body to the environment and fulfills a large variety of tasks in protecting the organism, regulating body temperature and water and energy balance, and serves as a sensory and immunological organ. The dermatogenetics research group aims at unravelling the molecular basis for genetic skin and hair diseases. Hans has particularly focused on diseases associated with impaired epidermal barrier function and disturbance of epidermal differentiation such as congenital ichthyosis. In an integrative approach, they provide molecular analysis of genetic skin diseases using gene panel and exome analysis with massively parallel sequencing and other genomics methods; investigate primary keratinocytes and have established 3D full-skin models with organotypic cell culture; and are using induced pluripotent stem (iPS) cells to generate disease- and patient-specific models for monogenic skin disorders. In an attempt to develop new therapy approaches for rare congenital skin diseases, they use nanoparticles for the delivery of drugs into the epidermis. They want to establish topical protein substitution as a new strategy for causative therapy. The development of disease models suitable for high-throughput studies will be instrumental for drug screening as well as protein substitution studies. Another research interest relates to the function of the dermal connective tissue. Dupuytren’s disease is the most frequent genetic disorder of connective tissues. It is a multifactorial disease with a strong genetic basis. Using a variety of approaches including genome-wide association studies (GWAS), exome sequencing, transcriptome analysis and studies of fibroblasts they want to elucidate the genetic and functional basis for Dupuytren’s disease and identify targets for novel therapies.

近期论文

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Becker, K., Siegert, S., Toliat, M., Du, J., Casper, R., Dolmans, G., Werker, P., Tinschert, S., Franke, A., Gieger, C., Strauch, K., Nothnagel, M., Nürnberg, P. and Hennies, H. (2016) ‘Meta-Analysis of Genome-Wide Association Studies and Network Analysis-Based Integration with Gene Expression Data Identify New Suggestive Loci and Unravel a Wnt-Centric Network Associated with Dupuytren’s Disease’ PLoS ONE . ISSN 1932-6203 Gruber, R., Rainer, G., Weiss, A., Udvardi, A., Thiele, H., Eckl, K., Schupart, R., Nürnberg, P., Zschocke, J., Schmuth, M., Volc-Platzer, B. and Hennies, H. (2016) ‘Epidermal barrier impairment associated with CYP4F22 mutations in autosomal recessive congenital ichthyosis’ British Journal of Dermatology . ISSN 0007-0963 Ammann, S., Schulz, A., Krägeloh-Mann, I., Dieckmann, N., Niethammer, K., Fuchs, S., Eckl, K., Plank, R., Werner, R., Altmüller, J., Thiele, H., Nürnberg, P., Bank, J., Strauss, A., von Bernuth, H., Zur Stadt, U., Grieve, S., Griffiths, G., Lehmberg, K., Hennies, H. and Ehl, S. (2016) ‘Mutations in AP3D1 associated with immunodeficiency and seizures define a new type of Hermansky-Pudlak syndrome’ Blood , 127 (8), pp. 997-1006. ISSN 0006-4971 Gruber, R., Börnchen, C., Rose, K., Daubmann, A., Volksdorf, T., Wladykowski, E., Vidal, Y., Peters, E., Danso, M., Bouwstra, J., Hennies, H., Moll, I., Schmuth, M. and Brandner, J. (2015) ‘Diverse regulation of claudin-1 and claudin-4 in atopic dermatitis’ American Journal of Physical Anthropology , 185 (10), pp. 2777-2789. ISSN 0002-9483 Hennies, H (2015) ‘All is balanced: inter-alpha-trypsin inhibitors as unseen extracellular matrix proteins in epidermal morphology and differentiation’ Experimental dermatology , 24 (9), pp. 661-662. ISSN 1600-0625 Becker, K., Tinschert, S., Lienert, A., Bleuler, P., Staub, F., Meinel, A., Rössler, J., Wach, W., Hoffmann, R., Kühnel, F., Damert, H., Nick, H., Spicher, R., Lenze, W., Langer, M., Nürnberg, P. and Hennies, H. (2015) ‘The importance of genetic susceptibility in Dupuytren's disease’ Clinical Genetics , 87 (5), pp. 483-487. ISSN 0009-9163 Gruber, R., Sugarman, J., Crumrine, D., Hupe, M., Mauro, T., Mauldin, E., Thyssen, J., Brandner, J., Hennies, H., Schmuth, M. and Elias, P. (2015) ‘Sebaceous gland, hair shaft, and epidermal barrier abnormalities in keratosis pilaris with and without filaggrin deficiency’ The American Journal of Pathology , 185 (4), pp. 1012-1021. ISSN 0002-9440 Moosbrugger-Martinz, V., Jalili, A., Schossig, A., Jahn-Bassler, K., Zschocke, J., Schmuth, M., Stingl, G., Eckl, K., Hennies, H. and Gruber, R. (2015) ‘Epidermal barrier abnormalities in exfoliative ichthyosis with a novel homozygous loss-of-function mutation in CSTA’ British Journal of Dermatology , 172 (6), pp. 1628-1632. ISSN 0007-0963 Witting, M., Molina, M., Obst, K., Plank, R., Eckl, K., Hennies, H., Calderon, M., Friess, W. and Hedtrich, S. (2015) ‘Thermosensitive dendritic polyglycerol-based nanogels for cutaneous delivery of biomacromolecules’ Nanomedicine , 11 (5), pp. 1179-1187. ISSN 1549-9634 Seifert, W., Kühnisch, J., Maritzen, T., Lommatzsch, S., Hennies, H., Bachmann, S., Horn, D. and Haucke, V. (2015) ‘Cohen syndrome-associated protein COH1 physically and functionally interacts with the small GTPase RAB6 at the Golgi complex and directs neurite outgrowth’ Journal of Biological Chemistry , 290 (6), pp. 3349-3358. ISSN 0021-9258 Alnasif, N., Zoschke, C., Fleige, E., Brodwolf, R., Boreham, A., Ruhl, E., Eckl, K., Merk, H., Hennies, H., Alexiev, U., Haag, R., Küchler, S. and Schäfer-Korting, M. (2014) ‘Penetration of normal, damaged and diseased skin--an in vitro study on dendritic core-multishell nanotransporters’ Journal of Controlled Release , 185, pp. 45-50. ISSN 0168-3659 Eckl, K., Weindl, G., Ackermann, K., Küchler, S., Casper, R., Radowski, M., Haag, R., Hennies, H. and Schäfer-Korting, M. (2014) ‘Increased cutaneous absorption reflects impaired barrier function of reconstructed skin models mimicking keratinisation disorders’ Experimental dermatology , 23 (4), pp. 286-288. ISSN 1600-0625 Schiller, S., Seebode, C., Hennies, H., Giehl, K. and Emmert, S. (2014) ‘Palmoplantar keratoderma (PPK): acquired and genetic causes of a not so rare disease’ Journal der Deutschen Dermatologischen Gesellschaft , 12 (9), pp. 781-788. ISSN 1610-0379 Eckl, K., Tidhar, R., Thiele, H., Oji, V., Hausser, I., Brodesser, S., Preil, M., Önal-Akan, A., Stock, F., Muller, D., Becker, K., Casper, R., Nürnberg, G., Altmüller, J., Nürnberg, P., Traupe, H., Futerman, A. and Hennies, H. (2013) ‘Impaired epidermal ceramide synthesis causes autosomal recessive congenital ichthyosis and reveals the importance of ceramide acyl chain length’ Journal of Investigative Dermatology , 133 (9), pp. 2202-2211. ISSN 0022-202X Nellen, R., Steijlen, P., Hennies, H., Fischer, J., Munro, C., Jonkman, M., van Steensel, M. and van Geel, M. (2013) ‘Haplotype analysis in western European patients with mal de Meleda: founder effect for the W15R mutation in the SLURP1 gene’ British Journal of Dermatology , 168 (6), pp. 1372-1374. ISSN 0007-0963 Pasternack, S., Refke, M., Paknia, E., Hennies, H., Franz, T., Schäfer, N., Fryer, A., van Steensel, M., Sweeney, E., Just, M., Grimm, C., Kruse, R., Ferrandiz, C., Nöthen, M., Fischer, U. and Betz, R. (2013) ‘Mutations in SNRPE, which encodes a core protein of the spliceosome, cause autosomal-dominant hypotrichosis simplex’ The American Journal of Human Genetics , 92 (1), pp. 81-87. ISSN 0002-9297 Shao, K., Koch, C., Gupta, M., Lin, Q., Lenz, M., Laufs, S., Denecke, B., Schmidt, M., Linke, M., Hennies, H., Hescheler, J., Zenke, M., Zechner, U., Saric, T. and Wagner, W. (2013) ‘Induced pluripotent mesenchymal stromal cell clones retain donor-derived differences in DNA methylation profiles’ Molecular Therapy—Nucleic Acids , 21 (1), pp. 240-250. ISSN 2162-2531 Jansen, P., van den Bogaard, E., Kersten, F., Oostendorp, C., van Vlijmen-Willems, I., Oji, V., Traupe, H., Hennies, H., Schalkwijk, J. and Zeeuwen, P. (2012) ‘Cystatin M/E knockdown by lentiviral delivery of shRNA impairs epidermal morphogenesis of human skin equivalents’ Experimental dermatology , 21 (11), pp. 889-891. ISSN 1600-0625 Aufenvenne, K., Rice, R., Hausser, I., Oji, V., Hennies, H., Rio, M., Traupe, H. and Larcher, F. (2012) ‘Long-term faithful recapitulation of transglutaminase 1-deficient lamellar ichthyosis in a skin-humanized mouse model, and insights from proteomic studies’ Journal of Investigative Dermatology , 132 (7), pp. 1918-1921. ISSN 0022-202X Hussain, M., Baig, S., Neumann, S., Nürnberg, G., Farooq, M., Ahmad, I., Alef, T., Hennies, H., Technau, M., Altmüller, J., Frommolt, P., Thiele, H., Noegel, A. and Nürnberg, P. (2012) ‘A truncating mutation of CEP135 causes primary microcephaly and disturbed centrosomal function’ The American Journal of Human Genetics , 90 (5), pp. 871-878. ISSN 0002-9297

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