gene targeting of the cysteine peptidase cathepsin h impairs lung surfactant in mice半胱氨酸肽酶的基因打靶小鼠组织蛋白酶h损害肺表面活性剂.pdfVIP

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gene targeting of the cysteine peptidase cathepsin h impairs lung surfactant in mice半胱氨酸肽酶的基因打靶小鼠组织蛋白酶h损害肺表面活性剂.pdf

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gene targeting of the cysteine peptidase cathepsin h impairs lung surfactant in mice半胱氨酸肽酶的基因打靶小鼠组织蛋白酶h损害肺表面活性剂

Gene Targeting of the Cysteine Peptidase Cathepsin H Impairs Lung Surfactant in Mice ¨ 1. 2. 3. 2,4,5. 6 Frank Buhling , Martin Kouadio , Caroline E. Chwieralski , Ursula Kern , Jens M. Hohlfeld , 2 2 2 2¤ 2,7 Nicole Klemm , Nicole Friedrichs , Wera Roth , Jan M. Deussing , Christoph Peters , Thomas Reinheckel2,7* 1 Institute of Laboratory Medicine, Carl-Thiem-Klinikum, Cottbus, Germany, 2 Institut of Molecular Medicine and Cell Research, Albert-Ludwigs-University, Freiburg, Germany, 3 Insitute of Molecular and Clinical Immunology, Otto-von-Guericke University, Magdeburg, Germany, 4 Faculty of Biology, Albert-Ludwigs-University, Freiburg, Germany, 5 Spemann Graduate School of Biology and Medicine (SGBM), Albert-Ludwigs-University, Freiburg, Germany, 6 Division of Immunology, Allergology and Airway Research, Fraunhofer Institute of Toxicology and Experimental Medicine, Hannover, Germany, 7 BIOSS Centre of Biological Signalling Studies, Albert-Ludwigs-University, Freiburg, Germany Abstract Background: The 11 human cysteine cathepsins are proteases mainly located in the endolysosomal compartment of all cells and within the exocytosis pathways of some secretory cell types. Cathepsin H (Ctsh) has amino- and endopeptidase activities. In vitro studies have demonstrated Ctsh involvement in the processing and secretion of the pulmonary surfactant protein B (SP-B). Furthermore, Ctsh is highly expressed in the secretory organelles of alveolar type II pneumocytes where the surfactant proteins are processed. Meth