Gene Symbol: Derl1
Description: derlin 1
Alias: RGD1311835, derlin-1, Der1-like domain family, member 1
Species: rat
Products:     Derl1

Top Publications

  1. Lilley B, Ploegh H. A membrane protein required for dislocation of misfolded proteins from the ER. Nature. 2004;429:834-40 pubmed
    ..We conclude that Derlin-1 is an important factor for the extraction of certain aberrantly folded proteins from the mammalian ER. ..
  2. Ye Y, Shibata Y, Yun C, Ron D, Rapoport T. A membrane protein complex mediates retro-translocation from the ER lumen into the cytosol. Nature. 2004;429:841-7 pubmed
    ..Derlin-1 interacts with US11, a virally encoded ER protein that specifically targets MHC class I heavy chains for export from the ER, as well as with VIMP, a novel membrane protein that recruits the p97 ATPase and its cofactor. ..
  3. Ye Y, Shibata Y, Kikkert M, van Voorden S, Wiertz E, Rapoport T. Recruitment of the p97 ATPase and ubiquitin ligases to the site of retrotranslocation at the endoplasmic reticulum membrane. Proc Natl Acad Sci U S A. 2005;102:14132-8 pubmed
  4. Oda Y, Okada T, Yoshida H, Kaufman R, Nagata K, Mori K. Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation. J Cell Biol. 2006;172:383-93 pubmed
    ..These findings indicate that Derlin-2 and -3 provide the missing link between EDEM and p97 in the process of degrading misfolded glycoproteins. ..
  5. Wang Q, Li L, Ye Y. Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3. J Cell Biol. 2006;174:963-71 pubmed
    ..We present evidence that atx3 may promote p97-associated deubiquitination to facilitate the transfer of polypeptides from p97 to the proteasome. ..
  6. Wang B, Heath Engel H, Zhang D, Nguyen N, Thomas D, Hanrahan J, et al. BAP31 interacts with Sec61 translocons and promotes retrotranslocation of CFTRDeltaF508 via the derlin-1 complex. Cell. 2008;133:1080-92 pubmed publisher
    ..Thus, BAP31 operates at early steps to deliver newly synthesized CFTRDeltaF508 to its degradation pathway...
  7. Schaheen B, Dang H, Fares H. Derlin-dependent accumulation of integral membrane proteins at cell surfaces. J Cell Sci. 2009;122:2228-39 pubmed publisher
  8. Chen S, Wu C, Lee Y, Tam K, Tsai Y, Liou J, et al. Caveolin-1 interacts with Derlin-1 and promotes ubiquitination and degradation of cyclooxygenase-2 via collaboration with p97 complex. J Biol Chem. 2013;288:33462-9 pubmed publisher
    ..Cav-1 may be a cofactor in the interaction of Derlin-1 and N-glycosylated COX-2 and may facilitate Derlin-1- and p97 complex-mediated COX-2 ubiquitination, retrotranslocation, and degradation. ..
  9. Ballar P, Zhong Y, Nagahama M, Tagaya M, Shen Y, Fang S. Identification of SVIP as an endogenous inhibitor of endoplasmic reticulum-associated degradation. J Biol Chem. 2007;282:33908-14 pubmed
    ..These results suggest that SVIP is an endogenous inhibitor of ERAD that acts through regulating the assembly of the gp78-p97/VCP-Derlin1 complex. ..

More Information


  1. Altier C, Garcia Caballero A, Simms B, You H, Chen L, Walcher J, et al. The Cav? subunit prevents RFP2-mediated ubiquitination and proteasomal degradation of L-type channels. Nat Neurosci. 2011;14:173-80 pubmed publisher
    ..Thus, Cav?? regulates the ubiquitination and stability of the calcium channel complex. ..
  2. El Khouri E, Le Pavec G, Toledano M, Delaunay Moisan A. RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR). J Biol Chem. 2013;288:31177-91 pubmed publisher
    ..Our data thus identify RNF185 and RNF5 as a novel E3 ligase module that is central to the control of CFTR degradation. ..
  3. Chen C, Malchus N, Hehn B, Stelzer W, Avci D, Langosch D, et al. Signal peptide peptidase functions in ERAD to cleave the unfolded protein response regulator XBP1u. EMBO J. 2014;33:2492-506 pubmed publisher
    ..Thus, we identify an ERAD complex that controls the abundance of XBP1u and thereby tunes signaling through the UPR. ..
  4. Kadowaki H, Nagai A, Maruyama T, Takami Y, Satrimafitrah P, Kato H, et al. Pre-emptive Quality Control Protects the ER from Protein Overload via the Proximity of ERAD Components and SRP. Cell Rep. 2015;13:944-56 pubmed publisher
    ..These findings demonstrate that Derlins-mediated substrate-specific rerouting and Bag6- and p97-mediated effective degradation contribute to the maintenance of ER homeostasis without the need for translocation. ..