1li1: Difference between revisions

[[Image:1li1.gif|left|200px]]<br /><applet load="1li1" size="350" color="white" frame="true" align="right" spinBox="true"  

Triple-helical collagen IV protomers associate through their N- and C-termini forming a three-dimensional network, which provides basement membranes with an anchoring scaffold and mechanical strength. The noncollagenous (NC1) domain of the C-terminal junction between two adjacent collagen IV protomers from human placenta was crystallized and its 1.9-A structure was solved by multiple anomalous diffraction (MAD) phasing. This hexameric NC1 particle is composed of two trimeric caps, which interact through a large planar interface. Each cap is formed by two alpha 1 fragments and one alpha 2 fragment with a similar previously uncharacterized fold, segmentally arranged around an axial tunnel. Each monomer chain folds into two structurally very similar subdomains, which each contain a finger-like hairpin loop that inserts into a six-stranded beta-sheet of the neighboring subdomain of the same or the adjacent chain. Thus each trimer forms a quite regular, but nonclassical, sixfold propeller. The trimer-trimer interaction is further stabilized by a previously uncharacterized type of covalent cross-link between the side chains of a Met and a Lys residue of the alpha 1 and alpha 2 chains from opposite trimers, explaining previous findings of nonreducible cross-links in NC1. This structure provides insights into NC1-related diseases such as Goodpasture and Alport syndromes.

Known diseases associated with this structure: Angiopathy, hereditary, with nephropathy, aneurysms, and muscle cramps OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120130 120130]], Brain small vessel disease with hemorrhage OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120130 120130]], Porencephaly OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120130 120130]]

1LI1 is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=ACT:'>ACT</scene> as [http://en.wikipedia.org/wiki/ligand ligand]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1LI1 OCA].  

[[Category: Bartunik, H D.]]

[[Category: Bourenkov, G P.]]

[[Category: Than, M E.]]

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