Search

Your search keyword '"Clemen C"' showing total 142 results
Start Over Author "Clemen C"
142 results on '"Clemen C"'

2. Aeroacoustic and Aerodynamic Importance of Unequal Rotor Rotation Speeds of a CROR

Author

Akkermans, R. A. D., Delfs, J. W., Márquez, C. O., Stuermer, A., Richter, C., Clemen, C., Caruelle, B., Omais, M., Boersma, Bendiks Jan, Series editor, Fujii, Kozo, Series editor, Haase, Werner, Series editor, Leschziner, Michael A., Series editor, Periaux, Jacques, Series editor, Pirozzoli, Sergio, Series editor, Rizzi, Arthur, Series editor, Roux, Bernard, Series editor, Shokin, Yurii I., Series editor, Dillmann, Andreas, editor, Heller, Gerd, editor, Krämer, Ewald, editor, Kreplin, Hans-Peter, editor, Nitsche, Wolfgang, editor, and Rist, Ulrich, editor

Published
2014
Full Text
View/download PDF

3. PRACTICAL EXAMPLES ON BIM-GIS INTEGRATION BASED ON SEMANTIC WEB TRIPLESTORES

Author

Schilling, S. and Clemen, C.

Subjects
Technology, Applied optics. Photonics, TA1-2040, Engineering (General). Civil engineering (General), TA1501-1820
Abstract

The integration of geodata and building models is one of the current challenges in the AECOO (architecture, engineering, construction, owner, operation) domain. Data from Building Information Models (BIM) and Geographical Information Systems (GIS) can’t be simply mapped 1:1 to each other because of their different domains. One possible approach is to convert all data in a domain-independent format and link them together in a semantic database. To demonstrate, how this data integration can be done in a federated database architecture, we utilize concepts of the semantic web, ontologies and the Resource Description Framework (RDF). It turns out, however, that traditional object-relational approaches provide more efficient access methods on geometrical representations than triplestores. Therefore we developed a hybrid approach with files, geodatabases and triplestores. This work-in-progress-paper (extend abstract) demonstrates our intermediate research results by practical examples and identifies opportunities and limitations of the hybrid approach.

Published
2022

17. Imbalances in protein homeostasis caused by mutant desmin

Author

Winter, L., Unger, A., Berwanger, C., Spoerrer, M., Tuerk, M., Chevessier, F., Strucksberg, K. -H., Schloetzer-Schrehardt, U., Wittig, I., Goldmann, W. H., Marcus, K., Linke, W. A., Clemen, C. S., Schroeder, R., Winter, L., Unger, A., Berwanger, C., Spoerrer, M., Tuerk, M., Chevessier, F., Strucksberg, K. -H., Schloetzer-Schrehardt, U., Wittig, I., Goldmann, W. H., Marcus, K., Linke, W. A., Clemen, C. S., and Schroeder, R.

Published
2019

19. P.79Malignant cardiac phenotype after pressure overload in autosomal-dominant desminopathies: Lessons from heterozygous R349P desmin knock-in mice

Author

Stöckigt, F., primary, Eichhorn, L., additional, Beiert, T., additional, Knappe, V., additional, Radecke, T., additional, Steinmetz, M., additional, Berwanger, C., additional, Nickenig, G., additional, Peeva, V., additional, Kunz, W., additional, Clemen, C., additional, Schröder, R., additional, and Schrickel, J., additional

Published
2019
Full Text
View/download PDF

23. Plectin‐related scapuloperoneal myopathy with treatment‐responsive myasthenic syndrome.

Author

Argente‐Escrig, H., Schultheis, D., Kamm, L., Schowalter, M., Thiel, C., Türk, M., Clemen, C. S., Muelas, N., Castañón, M. J., Wiche, G., Herrmann, H., Vilchez, J. J., and Schröder, R.

Subjects
NEMALINE myopathy, FACIOSCAPULOHUMERAL muscular dystrophy, MUSCLE diseases, CONGENITAL myasthenic syndromes, SYNDROMES, FLEXOR muscles
Abstract

The human plectin gene ( I PLEC i ) on chromosome 8q24 codes for a 4,684 amino acids large protein whose isoforms [1] are key for the functional and structural organization of filamentous cytoskeletal networks, thereby contributing to fundamental biomechanical properties of mechanical stress-bearing tissues [2]. Plectinopathies thus far comprise five autosomal-recessive entities, including epidermolysis bullosa simplex with muscular dystrophy (EBS-MD), EBS-MD with myasthenic syndrome (EBS-MD-MyS), limb girdle muscular dystrophy type 2Q, EBS with pyloric atresia, skin-only EBS and the autosomal-dominant variant EBS-Ogna [2,3]. A muscle MRI when aged 40 revealed pronounced fatty replacements of shoulder girdle muscles, the posterior compartment of thighs and the anterior compartment of his lower legs ( B Figure b 1C). [Extracted from the article]

Published
2021
Full Text
View/download PDF

24. Imbalances in protein homeostasis caused by mutant desmin

Author

Winter, L., primary, Unger, A., additional, Berwanger, C., additional, Spörrer, M., additional, Türk, M., additional, Chevessier, F., additional, Strucksberg, K.-H., additional, Schlötzer-Schrehardt, U., additional, Wittig, I., additional, Goldmann, W. H., additional, Marcus, K., additional, Linke, W. A., additional, Clemen, C. S., additional, and Schröder, R., additional

Published
2018
Full Text
View/download PDF

27. Mitochondrial pathology in desminopathies: lessons from man and mice

Author

Schröder, R, Winter, L, Wittig, I, Peeva, V, Chevessier, F, Kley, R, Marcus, K, Kornblum, C, Kunz, W, and Clemen, C

Subjects
ddc: 610, 610 Medical sciences, Medicine, musculoskeletal system
Abstract

Desminopathies are a group of familial and sporadic myopathies and cardiomyopathies that are caused by mutations in the human desmin gene on chromosome 2q35. They belong to the expanding group of protein aggregate myopathies and are currently classified in the subgroup of myofibrillar myopathies (MFMs).[for full text, please go to the a.m. URL], Joint-Meeting of the German Society for Neuropathology and Neuroanatomy (DGNN) and the Scandinavian Neuropathological Society (SNS)

Published
2016
Full Text
View/download PDF

32. DesR349P Mutation Results in Ultrastructural Disruptions and Compromise of Skeletal Muscle Biomechanics Already at Preclinical Stages in Young Mice before the Onset of Protein Aggregation

Author

Diermeier, S., Haug, M., Reischl, B., Buttgereit, A., Schuermann, S., Spoerrer, M., Goldmann, W. H., Fabry, B., Elhimine, F., Stehle, R., Pfitzer, G., Winter, L., Clemen, C., Schroeder, R., Friedrich, O., Diermeier, S., Haug, M., Reischl, B., Buttgereit, A., Schuermann, S., Spoerrer, M., Goldmann, W. H., Fabry, B., Elhimine, F., Stehle, R., Pfitzer, G., Winter, L., Clemen, C., Schroeder, R., and Friedrich, O.

Published
2016

33. AAV9-mediated gene transfer of desmin ameliorates cardiomyopathy in desmin-deficient mice

Author

Heckmann, M. B., Bauer, R., Jungmann, A., Winter, L., Rapti, K., Strucksberg, K-H, Clemen, C. S., Li, Z., Schroeder, R., Katus, H. A., Mueller, O. J., Heckmann, M. B., Bauer, R., Jungmann, A., Winter, L., Rapti, K., Strucksberg, K-H, Clemen, C. S., Li, Z., Schroeder, R., Katus, H. A., and Mueller, O. J.

Abstract

Mutations of the human desmin (DES) gene cause autosomal dominant and recessive myopathies affecting skeletal and cardiac muscle tissue. Desmin knockout mice (DES-KO), which develop progressive myopathy and cardiomyopathy, mirror rare human recessive desminopathies in which mutations on both DES alleles lead to a complete ablation of desmin protein expression. Here, we investigated whether an adeno-associated virus-mediated gene transfer of wild-type desmin cDNA (AAV-DES) attenuates cardiomyopathy in these mice. Our approach leads to a partial reconstitution of desmin protein expression and the de novo formation of the extrasarcomeric desmin-syncoilin network in cardiomyocytes of treated animals. This finding was accompanied by reduced fibrosis and heart weights and improved systolic left-ventricular function when compared with control vector-treated DES-KO mice. Since the re-expression of desmin protein in cardiomyocytes of DES-KO mice restores the extrasarcomeric desminsyncoilin cytoskeleton, attenuates the degree of cardiac hypertrophy and fibrosis, and improves contractile function, AAV-mediated desmin gene transfer may be a novel and promising therapeutic approach for patients with cardiomyopathy due to the complete lack of desmin protein expression.

Published
2016

34. Mula sa Kinaroroonan: Kapwa, Kapatiran and Bayan in Philippine Social Science

Author

Clemen C. Aquino

Subjects
General Social Sciences, Context (language use), Sociology, Social science, Social organization, Asian studies
Abstract

From the pioneering works of Enriquez, Covar and Salazar, which paved the way for the production of social science knowledge that is particularly meaningful and sensitive to Philippine culture and society, this paper explores the social significations of kapwa, kapatiran and bayan. In the context of the dominant influence of Western perspectives in Philippine social science, the understanding, appreciation and evaluation of their contributions continue to be an important undertaking. As a preliminary attempt to contribute to this discourse, a panlipunang pagbabanghay is offered as an approach or an outline for the analysis of Philippine social organization.

Published
2004

38. DesR349P Mutation Results in Ultrastructural Disruptions and Compromise of Skeletal Muscle Biomechanics Already at Preclinical Stages in Young Mice before the Onset of Protein Aggregation

Author

Diermeier, S., primary, Haug, M., additional, Reischl, B., additional, Buttgereit, A., additional, Schürmann, S., additional, Spörrer, M., additional, Goldmann, W.H., additional, Fabry, B., additional, Elhimine, F., additional, Stehle, R., additional, Pfitzer, G., additional, Winter, L., additional, Clemen, C., additional, Schröder, R., additional, and Friedrich, O., additional

Published
2016
Full Text
View/download PDF

39. DesR349P mutation results in ultrastructural disruptions and compromise of skeletal muscle biomechanics already at preclinical stages in young mice before the onset of protein aggregation.

Author

Diermeier, S., Iberl, J., Vetter, K., Haug, M., Reischl, B., Buttgereit, A., Schuermann, S., Spoerrer, M., Goldmann, W. H., Fabry, B., Elhimine, F., Stehle, R., Pfitzer, G., Winter, L., Clemen, C., Schroeder, R., Friedrich, O., Diermeier, S., Iberl, J., Vetter, K., Haug, M., Reischl, B., Buttgereit, A., Schuermann, S., Spoerrer, M., Goldmann, W. H., Fabry, B., Elhimine, F., Stehle, R., Pfitzer, G., Winter, L., Clemen, C., Schroeder, R., and Friedrich, O.

Published
2015

41. AAV-mediated cardiac gene transfer of wild-type desmin in mouse models for recessive desminopathies

Author

Ruppert, T., Heckmann, M. B., Rapti, K., Schultheis, D., Jungmann, A., Katus, H. A., Winter, L., Frey, N., Clemen, C. S., Schröder, R., and Müller, O. J.

Abstract

Mutations in the human desmin gene cause autosomal-dominant and recessive cardiomyopathies and myopathies with marked phenotypic variability. Here, we investigated the effects of adeno-associated virus (AAV)-mediated cardiac wild-type desmin expression in homozygous desmin knockout (DKO) and homozygous R349P desmin knockin (DKI) mice. These mice serve as disease models for two subforms of autosomal-recessive desminopathies, the former for the one with a complete lack of desmin protein and the latter for the one with solely mutant desmin protein expression in conjunction with protein aggregation pathology in striated muscle. Two-month-old mice were injected with either a single dose of 5?×?1012AAV9-hTNT2-mDes (AAV-Des) vector genomes or NaCl as control. One week after injection, mice were subjected to a forced swimming exercise protocol for 4 weeks. Cardiac function was monitored over a period of 15 month after injection and before the mice were sacrificed for biochemical and morphological analysis. AAV-mediated cardiac expression of wild-type desmin in both the homozygous DKO and DKI backgrounds reached levels seen in wild-type mice. Notably, AAV-Des treated DKO mice showed a regular subcellular distribution of desmin as well as a normalization of functional and morphological cardiac parameters. Treated DKI mice, however, showed an aberrant subcellular localization of desmin, unchanged functional cardiac parameters, and a trend toward an increased cardiac fibrosis. In conclusion, the effect of a high-dose AAV9-based desmin gene therapy is highly beneficial for the heart in DKO animals, but not in DKI mice.

Published
2019
Full Text
View/download PDF

43. Heteromeric p97/p97R155C complexes induce dominant negative changes in wild-type and autophagy 9-deficient Dictyostelium cells

Author

Arhzaouy, K, Strucksberg, KH, Tung, SM, Tangavelou, K, Stumpf, M, Faix, J, Schröder, R, Clemen, C, and Eichinger, L

Subjects
ddc: 610, 610 Medical sciences, Medicine, environment and public health
Abstract

p97 (VCP or valosin containing protein in mammals and Cdc48p in yeast) is a ubiquitously expressed and evolutionarily highly conserved hexameric member of the magnesium-dependent Walker P-loop AAA-ATPases. p97 has been associated with various essential cellular processes including ubiquitin-proteasome[for full text, please go to the a.m. URL], 57th Annual Meeting of the German Society for Neuropathology and Neuroanatomy (DGNN)

Published
2012
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results