DLL3 (E3J5R) Rabbit mAb

Notch signaling is activated upon engagement of the Notch receptor with its ligands, the DSL (Delta, Serrate, Lag2) proteins of single-pass type I membrane proteins. The DSL proteins contain multiple EGF-like repeats and a DSL domain that is required for binding to Notch (1,2). Five DSL proteins have been identified in mammals: Jagged1, Jagged2, Delta-like (DLL) 1, 3 and 4 (3). Ligand binding to the Notch receptor results in two sequential proteolytic cleavages of the receptor by the ADAM protease and the γ-secretase complex. The intracellular domain of Notch is released and then translocates to the nucleus where it activates transcription. Notch ligands may also be processed in a way similar to Notch, suggesting a bi-directional signaling through receptor-ligand interactions (4-6).Mutations in DLL3 cause spondylocostal dysostoses (SCD), a diverse group of disorders of axial skeletal malformation (7-10). 1.Wilson, A. and Radtke, F. (2006) FEBS Lett. 580, 2860-2868. 2.Hansson, E.M. et al. (2004) Semin. Cancer Biol. 14, 320-328. 3.Chiba, S. (2006) Stem Cells 24, 2437-2447. 4.Bland, C.E. et al. (2003) J. Biol. Chem. 278, 13607-13610. 5.Six, E. et al. (2003) Proc. Natl. Acad. Sci. USA 100, 7638-7643. 6.LaVoie, M.J. and Selkoe, D.J. (2003) J. Biol. Chem. 278, 34427-34437. 7.Whittock, N.V. et al. (2004) Clin Genet 66, 67-72. 8.Turnpenny, P.D. et al. (2003) J Med Genet 40, 333-9. 9.Bulman, M.P. et al. (2000) Nat Genet 24, 438-41. 10.Bonafé, L. et al. (2003) Clin Genet 64, 28-35.