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tisdag 19 juni 2018

ERAD kompleksin jäsenet derliinit. Niitä on kolme samantapaista proteiinia. .

Derlins are ERAD member proteins PROTEINS

Derliinien yleishahmo näkyy tästä hiivan derliinistä. Jokaisella on 4 transmembraania jaksoa.
























https://res.mdpi.com/genes/genes-04-00306/article_deploy/html/images/genes-04-00306-g003.png

GENES DERL1, DERL2 and DERL3. Pub Med information.

      • DERL1 (Kr.8q24.13) ) Names: DER-1, DER1, derlin-1

Preferred Names
derlin-1
Names
DERtrin-1
Der1-like domain family, member 1
degradation in endoplasmic reticulum protein-1
Expr. Thyroid, fat + 25 other tissues. Th he protein encoded by this gene is a member of the derlin family. Members of this family participate in the ER-associated degradation response and retrotranslocate misfolded or unfolded proteins from the ER lumen to the cytosol for proteasomal degradation. This protein recognizes substrate in the ER and works in a complex to retrotranslocate it across the ER membrane into the cytosol. This protein may select cystic fibrosis transmembrane conductance regulator protein (CFTR) for degradation as well as unfolded proteins in Alzheimer's disease. Alternative splicing results in multiple transcript variants that encode different protein isoforms. [provided by RefSeq, Aug 2012]
https://www.ncbi.nlm.nih.gov/gene/79139
Peptide structure and history: isoform b (1-231 amino acids)

Related articles in PubMed

  2. Derlin-1 is overexpressed in human colon cancer and promotes cancer cell proliferation. Tan X, et al. Mol Cell Biochem, 2015 Oct. PMID 26173415
  3. Derlin-1-immunopositive inclusions in patients with Alzheimer's disease. Honjo Y, et al. Neuroreport, 2012 Jul 11. PMID 22627700
  5. Derlin-1 is overexpressed on the tumor cell surface and enables antibody-mediated tumor targeting therapy. Ran Y, et al. Clin Cancer Res, 2008 Oct 15. PMID 18927294
See all (85) citations in PubMed
https://www.ncbi.nlm.nih.gov/gene?db=gene&report=generif&term=79139


      • DERL2 (Kr.17p13.2) ), CGI-101, DERtrtn-2, 7-LAN-1, F-LANa, FLANa, derlin-2

Preferred Names
derlin-2
Names
Der1-like domain family, member 2
carcinoma related
degradation in endoplasmic reticulum protein 2
https://www.ncbi.nlm.nih.gov/gene/51009
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 [PubMed 16449189]).[supplied by OMIM, Mar 2008] Expression. Ubiquitous expression in thyroid (RPKM 9.0), testis (RPKM 7.8) and 25 other

Peptide structure and history, derlin-2 isoform b


Related articles in PubMed

  5. Cloning and characterization of F-LANa, upregulated in human liver cancer. Ying H, et al. Biochem Biophys Res Commun, 2001 Aug 17. PMID 11500051
See all (39) citations in PubMed
Gene Rif
  1. 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.
  2. derlin2 functions with HRD1 in ERAD of certain substrates independent of their glycosylation status.
  3. Required for Polyoma virus infection.
  4. Findings indicate that Derlin-2 provides the missing link between EDEM and p97 in the process of degrading misfolded glycoproteins.
  5. 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.

      • DERL3 (Kr.22q11.23) C22orf14, IZP6, LLN2, Derlin-3

Preferred Names
derlin-3
Names
DERtrin 3
Der1-like domain family, member 3
degradation in endoplasmic reticulum protein 3
der1-like protein 3
The protein encoded by this gene belongs to the derlin family, and resides in the endoplasmic reticulum (ER). Proteins that are unfolded or misfolded in the ER must be refolded or degraded to maintain the homeostasis of the ER. This protein appears to be involved in the degradation of misfolded glycoproteins in the ER. Several alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Oct 2008] Expression
Broad expression in stomach (RPKM 12.2), duodenum (RPKM 11.1) and 22 other tissues See more

Peptide structure, history, isoform b ( altenative spliced from 3´)

https://www.ncbi.nlm.nih.gov/protein/NP_001002862.1

Related articles in PubMed

  5. A membrane protein required for dislocation of misfolded proteins from the ER. Lilley BN, et al. Nature, 2004 Jun 24. PMID 15215855
See all (21) citations in PubMed

Related articles in PubMed

  5. A membrane protein required for dislocation of misfolded proteins from the ER. Lilley BN, et al. Nature, 2004 Jun 24. PMID 15215855
See all (21) citations in PubMed

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

  3. Findings indicate that Derlin-3 provides the missing link between EDEM and p97 in the process of degrading misfolded glycoproteins.

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