Chaperones in sporadic inclusion body myositis
- Introduction Sporadic inclusion body myositis (sIBM) is characterized by myopathological features including rimmed vacuoles (RVs) and proteins associated with protein aggregation, autophagy, and inflammation. Previous proteomic studies of RV areas revealed an overrepresentation of several chaperones and subunits of the T‐complex protein 1 (TCP‐1), which is involved in prevention of protein aggregation. Methods To validate our proteomic findings, immunofluorescence analyses of selected chaperones and quantitative Western blot analysis of TCP‐1 proteins were performed in five sIBM patients and five healthy controls. Results Immunofluorescence studies confirmed increased immunoreactivity for VCP, UNC45B, GRP‐75, αB‐crystallin, LAMP‐2, Rab‐7a, and TCP‐1\(\alpha\) and TCP‐\(\Theta\) in RVs. Quantitative Western blot analysis revealed a significantly higher level of TCP‐1 in sIBM muscle tissue when compared with healthy controls. Discussion Our study findings validate new insights in protein quality control and degradation processes that seem to be relevant in sIBM. These data provide an important basis for future functional and therapeutic studies.
Author: | Anne-Katrin GüttschesORCiDGND, Frank JacobsenGND, Anja SchreinerGND, Janine Mertens-RillGND, Martin TegenthoffGND, Katrin Marcus-AlicORCiDGND, Matthias VorgerdORCiDGND, Rudolf Andre KleyGND |
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URN: | urn:nbn:de:hbz:294-79303 |
DOI: | https://doi.org/10.1002/mus.26742 |
Parent Title (English): | Muscle & nerve |
Subtitle (English): | validation of proteomic data |
Publisher: | Wiley-VCH Verlag |
Place of publication: | Weinheim |
Document Type: | Article |
Language: | English |
Date of Publication (online): | 2021/03/05 |
Date of first Publication: | 2019/10/23 |
Publishing Institution: | Ruhr-Universität Bochum, Universitätsbibliothek |
Tag: | T-complex protein 1; chaperones; inclusion body myositis; protein quality control; proteomics; quantitative Western blot analysis |
Volume: | 61 |
Issue: | 1 |
First Page: | 116 |
Last Page: | 121 |
Note: | Dieser Beitrag ist auf Grund des DEAL-Wiley-Vertrages frei zugänglich. |
Institutes/Facilities: | Medizinisches Proteom-Center |
Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil, Neurologische Universitätsklinik und Poliklinik | |
Dewey Decimal Classification: | Technik, Medizin, angewandte Wissenschaften / Medizin, Gesundheit |
open_access (DINI-Set): | open_access |
Licence (English): | Creative Commons - CC BY-NC-ND 4.0 - Attribution-NonCommercial-NoDerivatives 4.0 International |