2oo4
From Proteopedia
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Structure of LNR-HD (Negative Regulatory Region) from human Notch 2
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Overview
Notch receptors transmit signals between adjacent cells. Signaling is, initiated when ligand binding induces metalloprotease cleavage of Notch, within an extracellular negative regulatory region (NRR). We present here, the X-ray structure of the human NOTCH2 NRR, which adopts an autoinhibited, conformation. Extensive interdomain interactions within the NRR bury the, metalloprotease site, showing that a substantial conformational movement, is necessary to expose this site during activation by ligand., Leukemia-associated mutations in NOTCH1 probably release autoinhibition by, destabilizing the conserved hydrophobic core of the NRR.
Disease
Known disease associated with this structure: Alagille syndrome 2 OMIM:[600275]
About this Structure
2OO4 is a Single protein structure of sequence from Homo sapiens with CA, ZN and GOL as ligands. Full crystallographic information is available from OCA.
Reference
Structural basis for autoinhibition of Notch., Gordon WR, Vardar-Ulu D, Histen G, Sanchez-Irizarry C, Aster JC, Blacklow SC, Nat Struct Mol Biol. 2007 Apr;14(4):295-300. Epub 2007 Apr 1. PMID:17401372
Page seeded by OCA on Mon Nov 12 23:13:58 2007
