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From Proteopedia
Crystal structure of the CCM2 C-terminal Harmonin Homology Domain (HHD)
Structural highlights
DiseaseCCM2_HUMAN Hereditary cerebral cavernous malformation. The disease is caused by mutations affecting the gene represented in this entry. FunctionCCM2_HUMAN Component of the CCM signaling pathway which is a crucial regulator of heart and vessel formation and integrity. May act through the stabilization of endothelial cell junctions (By similarity). May function as a scaffold protein for MAP2K3-MAP3K3 signaling. Seems to play a major role in the modulation of MAP3K3-dependent p38 activation induced by hyperosmotic shock (By similarity). Publication Abstract from PubMedCerebral cavernous malformations (CCM) are neurovascular dysplasias affecting up to 0.5% of the population. Mutations in the CCM2 gene are associated with acquisition of CCM. We identify a previously uncharacterized domain at the C-terminus of CCM2 and determine its 1.9A resolution crystal structure. Because this domain is structurally homologous to the N-terminal domain of harmonin, we name it the CCM2 harmonin-homology domain or HHD. CCM2 HHD is observed in two conformations, and we employ analytical ultracentrifugation to test its oligomerization. Additionally, CCM2 HHD contains an unusually long 13-residue 3(10) helix. This study provides the first structural characterization of CCM2. STRUCTURED SUMMARY OF PROTEIN INTERACTIONS: CCM2binds to CCM3 by pull down (View interaction) CCM2 and CCM2bind by X-ray crystallography (View interaction) CCM2 and CCM2bind by molecular sieving (View interaction). Structural studies of cerebral cavernous malformations 2 (CCM2) reveal a folded helical domain at its C-terminus.,Fisher OS, Zhang R, Li X, Murphy JW, Demeler B, Boggon TJ FEBS Lett. 2013 Jan 31;587(3):272-7. doi: 10.1016/j.febslet.2012.12.011. Epub, 2012 Dec 22. PMID:23266514[1] From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine. References
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Categories: Homo sapiens | Large Structures | Boggon TJ | Fisher OS | Li X | Murphy JW | Zhang R
