Disease relevance of DERL2

• Increased expression of F-LANa was confirmed by Northern blot analysis in 10 of 14 (71%) cases of hepatocellular carcinomas [1].
• Cloning and characterization of F-LANa, upregulated in human liver cancer [1].
• 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 [2].
• Two novel IL2-dependent cell lines, DERL-2 and DERL-7, were established from a patient with hepatosplenic gammadelta T cell lymphoma [2].

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].

References