例如:"lncRNA", "apoptosis", "WRKY"

Proteasomal Degradation of Proinsulin Requires Derlin-2, HRD1 and p97.

PLoS ONE. 2015 Jun 24;10(6):e0128206. eCollection 2015
Hanneke Hoelen 1 , Arnaud Zaldumbide 2 , Wouter F van Leeuwen 1 , Ellen C W Torfs 1 , Marten A Engelse 3 , Chopie Hassan 4 , Robert Jan Lebbink 1 , Eelco J de Koning 3 , Maaike E Resssing 5 , Arnoud H de Ru 4 , Peter A van Veelen 4 , Rob C Hoeben 2 , Bart O Roep 4 , Emmanuel J H J Wiertz 1
Hanneke Hoelen 1 , Arnaud Zaldumbide 2 , Wouter F van Leeuwen 1 , Ellen C W Torfs 1 , Marten A Engelse 3 , Chopie Hassan 4 , Robert Jan Lebbink 1 , Eelco J de Koning 3 , Maaike E Resssing 5 , Arnoud H de Ru 4 , Peter A van Veelen 4 , Rob C Hoeben 2 , Bart O Roep 4 , Emmanuel J H J Wiertz 1
+ et al

[No authors listed]

Author information
  • 1 Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, The Netherlands.
  • 2 Department of Molecular Cell Biology, Leiden University Medical Center, Leiden, The Netherlands.
  • 3 Department of Nephrology, Leiden University Medical Center, Leiden, The Netherlands.
  • 4 Department of Immunohematology & Blood Transfusion, Leiden University Medical Center, Leiden, The Netherlands.
  • 5 Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Molecular Cell Biology, Leiden University Medical Center, Leiden, The Netherlands.
全文

摘要


Patients with type 1 diabetes (T1D) suffer from beta-cell destruction by CD8+ T-cells that have preproinsulin as an important target autoantigen. It is of great importance to understand the molecular mechanism underlying the processing of preproinsulin into these CD8+ T-cell epitopes. We therefore studied a pathway that may contribute to the production of these antigenic peptides: degradation of proinsulin via ER associated protein degradation (ERAD). Analysis of the MHC class I peptide ligandome confirmed the presentation of the most relevant MHC class I-restricted diabetogenic epitopes in our cells: the signal peptide-derived sequence A15-A25 and the insulin B-chain epitopes H29-A38 and H34-V42. We demonstrate that specific silencing of Derlin-2, p97 and HRD1 by shRNAs increases steady state levels of proinsulin. This indicates that these ERAD constituents are critically involved in proinsulin degradation and may therefore also play a role in subsequent antigen generation. These ERAD proteins therefore represent interesting targets for novel therapies aiming at the reduction and possibly also prevention of beta-cell directed auto-immune reactions in T1D.