1hfh
From Proteopedia
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SOLUTION STRUCTURE OF A PAIR OF COMPLEMENT MODULES BY NUCLEAR MAGNETIC RESONANCE
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Overview
A portion of human complement factor H spanning the 15th (H15) and 16th, (H16) of its 20 modules, has been expressed in a yeast vector and, subjected to structure determination in solution using two-dimensional, 1H-NMR. The structure of H15 is very similar to that already established, for the fifth module of factor H and H16, consistent with the view that, all such complement control (C-) modules share a common overall topology., In addition, the tertiary structures of the component modules of the, H15-16 pair are very similar to those of the modules when expressed, individually, implying that each folds entirely autonomously within intact, factor H. Aromatic residues in the third turn of H15 and the second turn, of H16, together with a leucine residue from the linker region, contribute, to a small intermodular interface. Comparatively few nuclear Overhauser, effects were observable between protons on different modules., Consequently, a wide range of angles of "twist" (131 (+/- 146) degrees, mean value (+/- 1 standard deviation)), i.e. rotation about the long axis, of one module with respect to the other, exists in the family of, structures generated on the basis of the experimental data. However, much, smaller variations occur in the two, orthogonal, angles (175 (+/- 12), degrees and 103 (+/- 6) degrees) that describe the "tilt". These, observations may suggest upper limits on the relative flexibility of the, two modules. Models were built to assess the outcome of applying such, restrictions to all the neighbours within a string of 20 C-modules, and, the resulting structures compare well with factor H as visualized by, electron microscopy.
Disease
Known diseases associated with this structure: Complement factor H deficiency OMIM:[134370], Factor H and factor H-like 1 OMIM:[134370], Hemolytic-uremic syndrome OMIM:[134370], Macular degeneration, age-related, 4 OMIM:[134370], Membranoproliferative glomerulonephritis with CFH deficiency OMIM:[134370]
About this Structure
1HFH is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Solution structure of a pair of complement modules by nuclear magnetic resonance., Barlow PN, Steinkasserer A, Norman DG, Kieffer B, Wiles AP, Sim RB, Campbell ID, J Mol Biol. 1993 Jul 5;232(1):268-84. PMID:8331663
Page seeded by OCA on Mon Nov 12 17:17:23 2007
