Professur für Neuropathologie

Adresse:
Schwabachanlage 6
91054 Erlangen


Forschungsprojekt(e)


Biomechanical changes of skeletal muscle in desminopathies - a novel approach to unravel the causes of progressive weakness in inherited muscle diseases
Prof. Wolfgang Goldmann; Prof. Dr. Rolf Schröder; Prof. Dr. Dr. Oliver Friedrich
(01.07.2014 - 30.06.2015)



Publikationen (Download BibTeX)

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Haug, M., Meyer, C., Reischl, B., Prölß, G., Vetter, K., Iberl, J.,... Friedrich, O. (2019). The MyoRobot technology discloses a premature biomechanical decay of skeletal muscle fiber bundles derived from R349P desminopathy mice. Scientific Reports, 9(1). https://dx.doi.org/10.1038/s41598-019-46723-6
Winter, L., Unger, A., Berwanger, C., Spörrer, M., Türk, M., Chevessier, F.,... Schröder, R. (2019). Imbalances in protein homeostasis caused by mutant desmin. Neuropathology and Applied Neurobiology, 45(5), 476-494. https://dx.doi.org/10.1111/nan.12516
Dittrich, S., Graf, E., Trollmann, R., Neudorf, U., Schara, U., Heilmann, A.,... Eichhorn, J. (2019). Effect and safety of treatment with ACE-inhibitor Enalapril and β-blocker metoprolol on the onset of left ventricular dysfunction in Duchenne muscular dystrophy - A randomized, double-blind, placebo-controlled trial. Orphanet Journal of Rare Diseases, 14(1). https://dx.doi.org/10.1186/s13023-019-1066-9
Minakaki, G., Canneva, F., Chevessier, F., Bode, F., Menges, S., Timotius, I.,... Klucken, J. (2019). Treadmill exercise intervention improves gait and postural control in alpha-synuclein mouse models without inducing cerebral autophagy. Behavioural Brain Research, 363, 199-215. https://dx.doi.org/10.1016/j.bbr.2018.11.035
Moreira, A., Loquai, C., Pfoefer, C., Kaehler, K.C., Knauss, S., Heppt, M.V.,... Heinzerling, L. (2019). Myositis and neuromuscular side-effects induced by immune checkpoint inhibitors. European journal of cancer, 106, 12-23. https://dx.doi.org/10.1016/j.ejca.2018.09.033
Spörrer, M., Prochnicki, A., Tölle, R.C., Nyström, A., Esser, P.R., Homberg, M.,... Kiritsi, D. (2019). Treatment of keratinocytes with 4-phenylbutyrate in epidermolysis bullosa: Lessons for therapies in keratin disorders. EBioMedicine. https://dx.doi.org/10.1016/j.ebiom.2019.04.062
Brackmann, F., Türk, M., Gratzki, N., Rompel, O., Jungbluth, H., Schröder, R., & Trollmann, R. (2018). Compound heterozygous RYR1 mutations in a preterm with arthrogryposis multiplex congenita and prenatal CNS bleeding. Neuromuscular Disorders, 28(1), 54-58. https://dx.doi.org/10.1016/j.nmd.2017.09.009
Kustermann, M., Manta, L., Paone, C., Kustermann, J., Lausser, L., Wiesner, C.,... Just, S. (2018). Loss of the novel Vcp (valosin containing protein) interactor Washc4 interferes with autophagy-mediated proteostasis in striated muscle and leads to myopathy in vivo. Autophagy, 14(11), 1911-1927. https://dx.doi.org/10.1080/15548627.2018.1491491
Song, L., Rijal, R., Karow, M., Stumpf, M., Hahn, O., Park, L.,... Eichinger, L. (2018). Expression of N471D strumpellin leads to defects in the endolysosomal system. Disease Models & Mechanisms, 11(9). https://dx.doi.org/10.1242/dmm.033449
Mengel, D., Librizzi, D., Schoser, B., Glaeser, D., Clemen, C.S., Dodel, R., & Schröder, R. (2018). Inclusion Body Myopathy, Paget's Disease, and Fronto-temporal Dementia: a VCP-related Multi-systemic Proteinopathy. Fortschritte der Neurologie, Psychiatrie, 86(7), 434-438. https://dx.doi.org/10.1055/s-0044-101033
Türk, M., Weber, I., Vogt-Ladner, G., Schröder, R., & Winterholler, M. (2018). Diaphragmatic dysfunction as the presenting symptom in neuromuscular disorders: A retrospective longitudinal study of etiology and outcome in 30 German patients. Neuromuscular Disorders, 28(6), 484-490. https://dx.doi.org/10.1016/j.nmd.2018.04.001
Delbridge, C., Türk, M., Agaimy, A., Winterholler, M., & Schröder, R. (2018). Perivascular hemosiderin deposits in human skeletal muscle tissue. Neuromuscular Disorders, 28(6), 538-539. https://dx.doi.org/10.1016/j.nmd.2018.04.004
Clemen, C.S., Winter, L., Strucksberg, K.-H., Berwanger, C., Türk, M., Kornblum, C.,... Schröder, R. (2018). The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice? Biochemical and Biophysical Research Communications, 503(4), 2770-2777. https://dx.doi.org/10.1016/j.bbrc.2018.08.038
Regensburger, M., Türk, M., Pagenstecher, A., Schröder, R., & Winkler, J. (2017). VCP-related multisystem proteinopathy presenting as early-onset Parkinson disease. Neurology, 89(7), 746-748. https://dx.doi.org/10.1212/WNL.0000000000004240
Türk, M., Schröder, R., Khuller, K., Hofmann, A., Berwanger, C., Ludolph, A.C.,... Clemen, C.S. (2017). Genetic analysis of VCP and WASH complex genes in a German cohort of sporadic ALS-FTD patients. Neurobiology of Aging, 56, 213.e1-213.e5. https://dx.doi.org/10.1016/j.neurobiolaging.2017.04.023
Diermeier, S., Buttgereit, A., Schürmann, S., Winter, L., Xu, H., Murphy, R.M.,... Friedrich, O. (2017). Preaged remodeling of myofibrillar cytoarchitecture in skeletal muscle expressing R349P mutant desmin. Neurobiology of Aging, 58, 77-87. https://dx.doi.org/10.1016/j.neurobiolaging.2017.06.001
Unger, A., Beckendorf, L., Boehme, P., Kley, R., Von Frieling-Salewsky, M., Lochmueller, H.,... Linke, W.A. (2017). Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function. Acta Neuropathologica Communications, 5(1), 72. https://dx.doi.org/10.1186/s40478-017-0474-0
Diermeier, S., Iberl, J., Vetter, K., Haug, M., Pollmann, C., Reischl, B.,... Friedrich, O. (2017). Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice. Scientific Reports, 7(1), 1391. https://dx.doi.org/10.1038/s41598-017-01485-x
Argente-Escrig, H., Gomez, N.M., Gomez, L., Türk, M., Thiel, C., Schröder, R., & Vilchez, J.J. (2017). Ephedrine and 3,4 diaminopyridine responsive myasthenic syndrome in plectin-related epidermolysis bullosa simplex with muscular dystrophy. (pp. 205-205).
Diermeier, S., Buttgereit, A., Clemen, C., Schröder, R., & Friedrich, O. (2016). Aging-related progressive skeletal muscle weakness in mutant DesR349P muscles: a matter of compromised cytoarchitecture. Acta Physiologica, 216.

Zuletzt aktualisiert 2019-24-04 um 10:29