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Systematic name YOL013C
Gene name HRD1
Aliases DER3
Feature type ORF, Verified
Coordinates Chr XV:303035..301380
Primary SGDID S000005373

Description of YOL013C: Ubiquitin-protein ligase; required for endoplasmic reticulum-associated degradation (ERAD) of misfolded proteins; genetically linked to the unfolded protein response (UPR); regulated through association with Hrd3p; contains an H2 ring finger; likely plays a general role in targeting proteins that persistently associate with and potentially obstruct the ER-localized translocon[1][2][3][4][5][6][7]


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  1. Bays NW, et al. (2001) Hrd1p/Der3p is a membrane-anchored ubiquitin ligase required for ER-associated degradation. Nat Cell Biol 3(1):24-9 SGD PMID 11146622
  2. Bordallo J, et al. (1998) Der3p/Hrd1p is required for endoplasmic reticulum-associated degradation of misfolded lumenal and integral membrane proteins. Mol Biol Cell 9(1):209-22 SGD PMID 9437001
  3. Deak PM and Wolf DH (2001) Membrane topology and function of Der3/Hrd1p as a ubiquitin-protein ligase (E3) involved in endoplasmic reticulum degradation. J Biol Chem 276(14):10663-9 SGD PMID 11139575
  4. Friedlander R, et al. (2000) A regulatory link between ER-associated protein degradation and the unfolded-protein response. Nat Cell Biol 2(7):379-84 SGD PMID 10878801
  5. Gardner RG, et al. (2000) Endoplasmic reticulum degradation requires lumen to cytosol signaling. Transmembrane control of Hrd1p by Hrd3p. J Cell Biol 151(1):69-82 SGD PMID 11018054
  6. Hampton RY, et al. (1996) Role of 26S proteasome and HRD genes in the degradation of 3-hydroxy-3-methylglutaryl-CoA reductase, an integral endoplasmic reticulum membrane protein. Mol Biol Cell 7(12):2029-44 SGD PMID 8970163
  7. Rubenstein EM, et al. (2012) Aberrant substrate engagement of the ER translocon triggers degradation by the Hrd1 ubiquitin ligase. J Cell Biol 197(6):761-73 SGD PMID 22689655

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