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Items: 1 to 20 of 61

1.

Imbalances in protein homeostasis caused by mutant desmin.

Winter L, Unger A, Berwanger C, Spörrer M, Türk M, Chevessier F, Strucksberg KH, Schlötzer-Schrehardt U, Wittig I, Goldmann WH, Marcus K, Linke WA, Clemen CS, Schröder R.

Neuropathol Appl Neurobiol. 2018 Sep 4. doi: 10.1111/nan.12516. [Epub ahead of print]

PMID:
30179276
2.

The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?

Clemen CS, Winter L, Strucksberg KH, Berwanger C, Türk M, Kornblum C, Florin A, Aguilar-Pimentel JA, Amarie OV, Becker L, Garrett L, Hans W, Moreth K, Neff F, Pingen L, Rathkolb B, Rácz I, Rozman J, Treise I, Fuchs H, Gailus-Durner V, de Angelis MH, Vorgerd M, Eichinger L, Schröder R.

Biochem Biophys Res Commun. 2018 Sep 18;503(4):2770-2777. doi: 10.1016/j.bbrc.2018.08.038. Epub 2018 Aug 9.

3.

Coronin 1C promotes triple-negative breast cancer invasiveness through regulation of MT1-MMP traffic and invadopodia function.

Castagnino A, Castro-Castro A, Irondelle M, Guichard A, Lodillinsky C, Fuhrmann L, Vacher S, Agüera-González S, Zagryazhskaya-Masson A, Romao M, El Kesrouani C, Noegel AA, Dubois T, Raposo G, Bear JE, Clemen CS, Vincent-Salomon A, Bièche I, Chavrier P.

Oncogene. 2018 Jul 31. doi: 10.1038/s41388-018-0422-x. [Epub ahead of print]

PMID:
30065298
4.

Expression of N471D strumpellin leads to defects in the endolysosomal system.

Song L, Rijal R, Karow M, Stumpf M, Hahn O, Park L, Insall R, Schröder R, Hofmann A, Clemen CS, Eichinger L.

Dis Model Mech. 2018 Sep 13;11(9). pii: dmm033449. doi: 10.1242/dmm.033449.

5.

[Inclusion Body Myopathy, Paget's Disease, and Fronto-temporal Dementia: a VCP-related Multi-systemic Proteinopathy].

Mengel D, Librizzi D, Schoser B, Gläser D, Clemen CS, Dodel R, Schröder R.

Fortschr Neurol Psychiatr. 2018 Jul;86(7):434-438. doi: 10.1055/s-0044-101033. Epub 2018 Jul 20. German.

PMID:
30029282
6.

Loss of the novel Vcp (valosin containing protein) interactor Washc4 interferes with autophagy-mediated proteostasis in striated muscle and leads to myopathy in vivo.

Kustermann M, Manta L, Paone C, Kustermann J, Lausser L, Wiesner C, Eichinger L, Clemen CS, Schröder R, Kestler HA, Sandri M, Rottbauer W, Just S.

Autophagy. 2018;14(11):1911-1927. doi: 10.1080/15548627.2018.1491491. Epub 2018 Aug 16.

PMID:
30010465
7.

Correction: Heteromeric p97/p97R155C Complexes Induce Dominant Negative Changes in Wild-Type and Autophagy 9-Deficient Dictyostelium strains.

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

PLoS One. 2018 Jun 18;13(6):e0199548. doi: 10.1371/journal.pone.0199548. eCollection 2018.

8.

Aging-related mitochondrial dysfunction facilitates the occurrence of serious arrhythmia after myocardial infarction.

Stöckigt F, Beiert T, Knappe V, Baris OR, Wiesner RJ, Clemen CS, Nickenig G, Andrié RP, Schrickel JW.

Biochem Biophys Res Commun. 2017 Nov 4;493(1):604-610. doi: 10.1016/j.bbrc.2017.08.145. Epub 2017 Sep 1.

PMID:
28867191
9.

Preaged remodeling of myofibrillar cytoarchitecture in skeletal muscle expressing R349P mutant desmin.

Diermeier S, Buttgereit A, Schürmann S, Winter L, Xu H, Murphy RM, Clemen CS, Schröder R, Friedrich O.

Neurobiol Aging. 2017 Oct;58:77-87. doi: 10.1016/j.neurobiolaging.2017.06.001. Epub 2017 Jun 13.

PMID:
28715662
10.

Genetic analysis of VCP and WASH complex genes in a German cohort of sporadic ALS-FTD patients.

Türk M, Schröder R, Khuller K, Hofmann A, Berwanger C, Ludolph AC, Dekomien G, Müller K, Weishaupt JH, Thiel CT, Clemen CS.

Neurobiol Aging. 2017 Aug;56:213.e1-213.e5. doi: 10.1016/j.neurobiolaging.2017.04.023. Epub 2017 May 3.

PMID:
28551275
11.

Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice.

Diermeier S, Iberl J, Vetter K, Haug M, Pollmann C, Reischl B, Buttgereit A, Schürmann S, Spörrer M, Goldmann WH, Fabry B, Elhamine F, Stehle R, Pfitzer G, Winter L, Clemen CS, Herrmann H, Schröder R, Friedrich O.

Sci Rep. 2017 May 3;7(1):1391. doi: 10.1038/s41598-017-01485-x.

12.

The Power of Human Protective Modifiers: PLS3 and CORO1C Unravel Impaired Endocytosis in Spinal Muscular Atrophy and Rescue SMA Phenotype.

Hosseinibarkooie S, Peters M, Torres-Benito L, Rastetter RH, Hupperich K, Hoffmann A, Mendoza-Ferreira N, Kaczmarek A, Janzen E, Milbradt J, Lamkemeyer T, Rigo F, Bennett CF, Guschlbauer C, Büschges A, Hammerschmidt M, Riessland M, Kye MJ, Clemen CS, Wirth B.

Am J Hum Genet. 2016 Sep 1;99(3):647-665. doi: 10.1016/j.ajhg.2016.07.014. Epub 2016 Aug 4.

13.

Neuromuscular endplate pathology in recessive desminopathies: Lessons from man and mice.

Durmuş H, Ayhan Ö, Çırak S, Deymeer F, Parman Y, Franke A, Eiber N, Chevessier F, Schlötzer-Schrehardt U, Clemen CS, Hashemolhosseini S, Schröder R, Hemmrich-Stanisak G, Tolun A, Serdaroğlu-Oflazer P.

Neurology. 2016 Aug 23;87(8):799-805. doi: 10.1212/WNL.0000000000003004. Epub 2016 Jul 20.

PMID:
27440146
14.

Mutant desmin substantially perturbs mitochondrial morphology, function and maintenance in skeletal muscle tissue.

Winter L, Wittig I, Peeva V, Eggers B, Heidler J, Chevessier F, Kley RA, Barkovits K, Strecker V, Berwanger C, Herrmann H, Marcus K, Kornblum C, Kunz WS, Schröder R, Clemen CS.

Acta Neuropathol. 2016 Sep;132(3):453-73. doi: 10.1007/s00401-016-1592-7. Epub 2016 Jul 8.

15.

Coronin 1C-free primary mouse fibroblasts exhibit robust rearrangements in the orientation of actin filaments, microtubules and intermediate filaments.

Behrens J, Solga R, Ziemann A, Rastetter RH, Berwanger C, Herrmann H, Noegel AA, Clemen CS.

Eur J Cell Biol. 2016 Aug;95(8):239-51. doi: 10.1016/j.ejcb.2016.04.004. Epub 2016 May 4.

16.

Novel Coronin7 interactions with Cdc42 and N-WASP regulate actin organization and Golgi morphology.

Bhattacharya K, Swaminathan K, Peche VS, Clemen CS, Knyphausen P, Lammers M, Noegel AA, Rastetter RH.

Sci Rep. 2016 May 4;6:25411. doi: 10.1038/srep25411.

17.

Mutant p97 exhibits species-specific changes of its ATPase activity and compromises the UBXD9-mediated monomerisation of p97 hexamers.

Rijal R, Arhzaouy K, Strucksberg KH, Cross M, Hofmann A, Schröder R, Clemen CS, Eichinger L.

Eur J Cell Biol. 2016 Jun-Jul;95(6-7):195-207. doi: 10.1016/j.ejcb.2016.03.004. Epub 2016 Apr 7.

18.

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

Heckmann MB, Bauer R, Jungmann A, Winter L, Rapti K, Strucksberg KH, Clemen CS, Li Z, Schröder R, Katus HA, Müller OJ.

Gene Ther. 2016 Aug;23(8-9):673-9. doi: 10.1038/gt.2016.40. Epub 2016 Apr 21.

19.

Coronin 2A (CRN5) expression is associated with colorectal adenoma-adenocarcinoma sequence and oncogenic signalling.

Rastetter RH, Blömacher M, Drebber U, Marko M, Behrens J, Solga R, Hojeili S, Bhattacharya K, Wunderlich CM, Wunderlich FT, Odenthal M, Ziemann A, Eichinger L, Clemen CS.

BMC Cancer. 2015 Sep 15;15:638. doi: 10.1186/s12885-015-1645-7.

20.

VCP and PSMF1: Antagonistic regulators of proteasome activity.

Clemen CS, Marko M, Strucksberg KH, Behrens J, Wittig I, Gärtner L, Winter L, Chevessier F, Matthias J, Türk M, Tangavelou K, Schütz J, Arhzaouy K, Klopffleisch K, Hanisch FG, Rottbauer W, Blümcke I, Just S, Eichinger L, Hofmann A, Schröder R.

Biochem Biophys Res Commun. 2015 Aug 7;463(4):1210-7. doi: 10.1016/j.bbrc.2015.06.086. Epub 2015 Jun 15.

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