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Gene Review

DERL2  -  derlin 2

Homo sapiens

Synonyms: CGI-101, DER2, DERtrin-2, Degradation in endoplasmic reticulum protein 2, Der1-like protein 2, ...
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Disease relevance of DERL2


High impact information on DERL2

  • Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation [3].
  • Overexpression of Derlin-2 or -3 accelerated degradation of misfolded glycoprotein, whereas their knockdown blocked degradation [3].
  • We demonstrate here that Derlin-2 is an ER-resident protein that, similar to Derlin-1, participates in the degradation of proteins from the ER [4].
  • In addition, DERL-2 showed 5q+ in all metaphases analyzed, while DERL-7 revealed loss of one chromosome 4 [2].
  • By microdissection we have generated a painting probe from the mother's abnormal short arm of chromosome 2 (der2p probe) [5].

Biological context of DERL2

  • Mammalian genomes encode two additional, related proteins (Derlin-2 and Derlin-3) [4].
  • Simultaneous mutagenesis of the two half-sections, DER1 and DER2, of the DER sequence was required for derepression suggesting participation of multiple proteins in the DER-dependent transcriptional repression [6].
  • Human F-LANa gene maps to chromosome 17p at D17S1828-D17S786, spans at least 11.8 kb, and contains 7 exons [1].
  • In addition, F-LANa antisense oligonucleotide suppressed F-LANa expression in human hepatocellular carcinoma BEL-7404 cells and significantly inhibited cell growth [1].


  1. Cloning and characterization of F-LANa, upregulated in human liver cancer. Ying, H., Yu, Y., Xu, Y. Biochem. Biophys. Res. Commun. (2001) [Pubmed]
  2. Characterization of two novel cell lines, DERL-2 (CD56+/CD3+/Tcry5+) and DERL-7 (CD56+/CD3-/TCRgammadelta-), derived from a single patient with CD56+ non-Hodgkin's lymphoma. Di Noto, R., Pane, F., Camera, A., Luciano, L., Barone, M., Lo Pardo, C., Boccuni, P., Intrieri, M., Izzo, B., Villa, M.R., Macrí, M., Rotoli, B., Sacchetti, L., Salvatore, F., Del Vecchio, L. Leukemia (2001) [Pubmed]
  3. 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]
  4. 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]
  5. Microdissection of chromosome 2--between-arm intrachromosomal insertion. Friedrich, U., Houman, M., Sandgaard, J., Rosgaard, A., Sunde, L. Eur. J. Hum. Genet. (2000) [Pubmed]
  6. Negative transcriptional modulation and silencing of the bi-exonic Rnf35 gene in the preimplantation embryo. Binding of the CCAAT-displacement protein/Cux to the untranslated exon 1 sequence. Huang, C.J., Chang, J.G., Wu, S.C., Choo, K.B. J. Biol. Chem. (2005) [Pubmed]
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