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Gene Review

DERL3  -  derlin 3

Homo sapiens

Synonyms: C22orf14, DER3, DERtrin-3, Degradation in endoplasmic reticulum protein 3, Der1-like protein 3, ...
 
 
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High impact information on DERL3

  • Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation [1].
  • Partial trisomy 11,46,XX,-3,-20, + der3, + der20,t(3:11:20), resulting from a complex maternal rearrangement of chromosomes 3, 11, 20 [2].
  • Notably, another ER transmembrane E3, Hrd1 (also known as Der3), cannot localize efficiently to the inner nuclear membrane [3].
  • Cells of long-term cultures GER and STAH exhibited a consistent translocation t(2;18), a trisomy 7, and two consistent marker chromosomes der3 and 5p+, respectively [4].
 

Biological context of DERL3

References

  1. Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation. Oda, Y., Okada, T., Yoshida, H., Kaufman, R.J., Nagata, K., Mori, K. J. Cell Biol. (2006) [Pubmed]
  2. Partial trisomy 11,46,XX,-3,-20, + der3, + der20,t(3:11:20), resulting from a complex maternal rearrangement of chromosomes 3, 11, 20. Palmer, C.G., Poland, C., Reed, T., Kojetin, J. Hum. Genet. (1976) [Pubmed]
  3. Spatially regulated ubiquitin ligation by an ER/nuclear membrane ligase. Deng, M., Hochstrasser, M. Nature (2006) [Pubmed]
  4. Biologic and cytogenetic characterization of three human medullary thyroid carcinomas in culture. Pfragner, R., Wirnsberger, G., Behmel, A., Niederle, B., Längle, F., Roka, R., Mandl, A., Pürstner, P., Auner, J., Tatzber, F. Henry Ford Hosp. Med. J. (1992) [Pubmed]
  5. Multiprotein complexes that link dislocation, ubiquitination, and extraction of misfolded proteins from the endoplasmic reticulum membrane. Lilley, B.N., Ploegh, H.L. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
 
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