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Human Polyclonal DERL2 Primary Antibody for IHC - ABIN965991
Lilley, Ploegh: A membrane protein required for dislocation of misfolded proteins from the ER. in Nature 2004
Show all 3 Pubmed References
Derlin-2 is part of a protein quality control mechanism that can rescue glomerular injury attributable to impaired protein folding pathways in the endoplasmic reticulum.
specific silencing of Derlin-2, p97 and HRD1 by shRNAs increases steady state levels of proinsulin. these ERAD constituents are critically involved in proinsulin degradation and may therefore also play a role in subsequent antigen generation.
derlin2 functions with HRD1 in ERAD of certain substrates independent of their glycosylation status.
Derlin-2 forms a robust multiprotein complex with the p97 AAA ATPase as well as the mammalian orthologs of the yeast Hrd1p/Hrd3p ubiquitin-ligase complex, and participates in the degradation of proteins from the ER.
Findings indicate that Derlin-2 provides the missing link between EDEM and p97 in the process of degrading misfolded glycoproteins.
Required for Polyoma virus infection.
Whole-body deletion of derlin-2 leads to perinatal lethality and skeletal abnormalities.
Proteins that are unfolded or misfolded in the endoplasmic reticulum (ER) must be refolded or degraded to maintain the homeostasis of the ER. DERL2 is involved in the degradation of misfolded glycoproteins in the ER (Oda et al., 2006
, Der1-like domain family, member 2
, carcinoma related
, degradation in endoplasmic reticulum protein 2
, der1-like protein 2
, derlin 2