Endoplasmic Reticulum-Associated Degradation (ERAD) of Misfolded Glycoproteins and Mutant P23H Rhodopsin in Photoreceptor Cells

• Aebi M, Bernasconi R, Clerc S et al (2009) N-glycan structures: recognition and processing in the ER. Trends Biochem Sci 35:74–82

  Article  PubMed  Google Scholar 

• Anukanth A, Khorana HG (1994) Structure and function in rhodopsin. Requirements of a specific structure for the intradiscal domain. J Biol Chem 269:19738–19744

  PubMed  CAS  Google Scholar 

• Bays NW, Wilhovsky SK, Goradia A et al (2001) HRD4/NPL4 is required for the proteasomal processing of ubiquitinated ER proteins. Mol Biol Cell 12:4114–4128

  PubMed  CAS  Google Scholar 

• Bernasconi R, Galli C, Calanca V et al (2010) Stringent requirement for HRD1, SEL1L, and OS-9/XTP3-B for disposal of ERAD-LS substrates. J Cell Biol 188:223–235

  Article  PubMed  CAS  Google Scholar 

• Blom D, Hirsch C, Stern P et al (2004) A glycosylated type I membrane protein becomes cytosolic when peptide: N-glycanase is compromised. EMBO J 23:650–658

  Article  PubMed  CAS  Google Scholar 

• Blond-Elguindi S, Cwirla SE, Dower WJ et al (1993) Affinity panning of a library of peptides displayed on bacteriophages reveals the binding specificity of BiP. Cell 75:717–728

  Article  PubMed  CAS  Google Scholar 

• Braun S, Matuschewski K, Rape M et al (2002) Role of the ubiquitin-selective CDC48(UFD1/NPL4 )chaperone (segregase) in ERAD of OLE1 and other substrates. EMBO J 21:615–621

  Article  PubMed  CAS  Google Scholar 

• Christianson JC, Shaler TA, Tyler RE et al (2008) OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD. Nat Cell Biol 10:272–282

  Article  PubMed  CAS  Google Scholar 

• Ellgaard L (2004) Catalysis of disulphide bond formation in the endoplasmic reticulum. Biochem Soc Trans 32:663–667

  Article  PubMed  CAS  Google Scholar 

• Fagioli C, Sitia R (2001) Glycoprotein quality control in the endoplasmic reticulum. Mannose trimming by endoplasmic reticulum mannosidase I times the proteasomal degradation of unassembled immunoglobulin subunits. J Biol Chem 276:12885–12892

  Article  PubMed  CAS  Google Scholar 

• Finley D (2009) Recognition and processing of ubiquitin-protein conjugates by the proteasome. Annu Rev Biochem 78:477–513

  Article  PubMed  CAS  Google Scholar 

• Griciuc A, Aron L, Piccoli G et al (2010a) Clearance of Rhodopsin (P23H) aggregates requires the ERAD effector VCP. Biochim Biophys Acta 1803:424–434

  Article  PubMed  CAS  Google Scholar 

• Griciuc A, Aron L, Roux MJ et al (2010b) Inactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in Drosophila. PLoS Genet 6

  Google Scholar 

• Groll M, Ditzel L, Lowe J et al (1997) Structure of 20S proteasome from yeast at 2.4 A resolution. Nature 386:463–471

  Article  PubMed  CAS  Google Scholar 

• Hendershot LM, Wei JY, Gaut JR et al (1995) In vivo expression of mammalian BiP ATPase mutants causes disruption of the endoplasmic reticulum. Mol Biol Cell 6:283–296

  PubMed  CAS  Google Scholar 

• Hirao K, Natsuka Y, Tamura T et al (2006) EDEM3, a soluble EDEM homolog, enhances glycoprotein endoplasmic reticulum-associated degradation and mannose trimming. J Biol Chem 281:9650–9658

  Article  PubMed  CAS  Google Scholar 

• Hosokawa N, Kamiya Y, Kato K (2010a) The role of MRH domain-containing lectins in ERAD. Glycobiology 20:651–660

  Article  PubMed  CAS  Google Scholar 

• Hosokawa N, Wada I, Nagasawa K et al (2008) Human XTP3-B forms an endoplasmic reticulum quality control scaffold with the HRD1-SEL1L ubiquitin ligase complex and BiP. J Biol Chem 283:20914–20924

  Article  PubMed  CAS  Google Scholar 

• Hosokawa N, Tremblay LO, Sleno B et al (2010b) EDEM1 accelerates the trimming of alpha1,2-linked mannose on the C branch of N-glycans. Glycobiology 20:567–575

  Article  PubMed  CAS  Google Scholar 

• Illing ME, Rajan RS, Bence NF et al (2002) A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system. J Biol Chem 277:34150–34160

  Article  PubMed  CAS  Google Scholar 

• Jarosch E, Taxis C, Volkwein C et al (2002) Protein dislocation from the ER requires polyubiquitination and the AAA-ATPase Cdc48. Nat Cell Biol 4:134–139

  Article  PubMed  CAS  Google Scholar 

• Kang MJ, Ryoo HD (2009) Suppression of retinal degeneration in Drosophila by stimulation of ER-associated degradation. Proc Natl Acad Sci USA 106:17043–17048

  Article  PubMed  CAS  Google Scholar 

• Kosmaoglou M, Kanuga N, Aguila M et al (2009) A dual role for EDEM1 in the processing of rhodopsin. J Cell Sci 122:4465–4472

  Article  PubMed  CAS  Google Scholar 

• Lilley BN, Ploegh HL (2005) Multiprotein complexes that link dislocation, ubiquitination, and extraction of misfolded proteins from the endoplasmic reticulum membrane. Proc Natl Acad Sci USA 102:14296–14301

  Article  PubMed  CAS  Google Scholar 

• Lippincott-Schwartz J, Bonifacino JS, Yuan LC et al (1988) Degradation from the endoplasmic reticulum: disposing of newly synthesized proteins. Cell 54:209–220

  Article  PubMed  CAS  Google Scholar 

• Mast SW, Diekman K, Karaveg K et al (2005) Human EDEM2, a novel homolog of family 47 glycosidases, is involved in ER-associated degradation of glycoproteins. Glycobiology 15:421–436

  Article  PubMed  CAS  Google Scholar 

• Misaghi S, Pacold ME, Blom D et al (2004) Using a small molecule inhibitor of peptide: N-glycanase to probe its role in glycoprotein turnover. Chem Biol 11:1677–1687

  Article  PubMed  CAS  Google Scholar 

• Molinari M, Calanca V, Galli C et al (2003) Role of EDEM in the release of misfolded glycoproteins from the calnexin cycle. Science 299:1397–1400

  Article  PubMed  CAS  Google Scholar 

• Mueller B, Klemm EJ, Spooner E et al (2008) SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins. Proc Natl Acad Sci USA 105:12325–12330

  Article  PubMed  CAS  Google Scholar 

• Noorwez SM, Sama RR, Kaushal S (2009) Calnexin improves the folding efficiency of mutant rhodopsin in the presence of pharmacological chaperone 11-cis-retinal. J Biol Chem 284:33333–33342

  Article  PubMed  CAS  Google Scholar 

• Oda Y, Hosokawa N, Wada I et al (2003) EDEM as an acceptor of terminally misfolded glycoproteins released from calnexin. Science 299:1394–1397

  Article  PubMed  CAS  Google Scholar 

• Oliver JD, Roderick HL, Llewellyn DH et al (1999) ERp57 functions as a subunit of specific complexes formed with the ER lectins calreticulin and calnexin. Mol Biol Cell 10:2573–2582

  PubMed  CAS  Google Scholar 

• Peterson JR, Ora A, Van PN et al (1995) Transient, lectin-like association of calreticulin with folding intermediates of cellular and viral glycoproteins. Mol Biol Cell 6:1173–1184

  PubMed  CAS  Google Scholar 

• Richly H, Rape M, Braun S et al (2005) A series of ubiquitin binding factors connects CDC48/p97 to substrate multiubiquitylation and proteasomal targeting. Cell 120:73–84

  Article  PubMed  CAS  Google Scholar 

• Saliba RS, Munro PM, Luthert PJ et al (2002) The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation. J Cell Sci 115:2907–2918

  PubMed  CAS  Google Scholar 

• Sung CH, Schneider BG, Agarwal N et al (1991) Functional heterogeneity of mutant rhodopsins responsible for autosomal dominant retinitis pigmentosa. Proc Natl Acad Sci USA 88:8840–8844

  Article  PubMed  CAS  Google Scholar 

• Suzuki T, Park H, Kwofie MA et al (2001) Rad23 provides a link between the Png1 deglycosylating enzyme and the 26S proteasome in yeast. J Biol Chem 276:21601–21607

  Article  PubMed  CAS  Google Scholar 

• Trombetta ES, Helenius A (2000) Conformational requirements for glycoprotein reglucosylation in the endoplasmic reticulum. J Cell Biol 148:1123–1129

  Article  PubMed  CAS  Google Scholar 

• Trombetta ES, Parodi AJ (2003) Quality control and protein folding in the secretory pathway. Annu Rev Cell Dev Biol 19:649–676

  Article  PubMed  CAS  Google Scholar 

• Ushioda R, Hoseki J, Araki K et al (2008) ERdj5 is required as a disulfide reductase for degradation of misfolded proteins in the ER. Science 321:569–572

  Article  PubMed  CAS  Google Scholar 

• Wada I, Rindress D, Cameron PH et al (1991) SSR alpha and associated calnexin are major calcium binding proteins of the endoplasmic reticulum membrane. J Biol Chem 266:19599–19610

  PubMed  CAS  Google Scholar 

• Weissman AM (2001) Themes and variations on ubiquitylation. Nat Rev Mol Cell Biol 2:169–178

  Article  PubMed  CAS  Google Scholar 

• Ye Y, Meyer HH, Rapoport TA (2001) The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol. Nature 414:652–656

  Article  PubMed  CAS  Google Scholar 

• Zhang X, Shaw A, Bates PA et al (2000) Structure of the AAA ATPase p97. Mol Cell 6:1473–1484

  Article  PubMed  CAS  Google Scholar 

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