1k13 | pdb_00001k13

Theoretical Model: The protein structure described on this page was determined theoretically, and hence should be interpreted with caution.

SEVEN TANDEM LEUCINE-RICH REPEATS OF HUMAN GLYCOPROTEIN 1B ALPHA

spinqualitylabelsall models 1k13 Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights For a guided tour on the structure components use FirstGlance. Resources: FirstGlance, PDBsum, ProSAT Publication Abstract from PubMed Platelet glycoprotein (GP)Ib-IX-V mediates von Willebrand Factor (vWF)-dependent adhesion to vascular subendothelium at high shear in (patho)physiological thrombus formation. The ligand-binding domain of GPIb-IX-V is within the N-terminal 282 residues of GPIb alpha, that contains seven tandem leucine-rich repeats (Leu36-Ala200). Repeats 2-4 are critical for vWF binding. In this study, we have built molecular models of the seven leucine-rich repeats of human, canine and mouse GPIb alpha, providing novel insights into the species-specific interaction between human vWF and its receptor. Interestingly, a major difference between the models was a large negatively charged patch on the concave face of human, but not canine, repeats 2-4. In addition, five individual mutations within the leucine-rich repeats of GPIb alpha associated with the bleeding disorder Bernard-Soulier syndrome, that result in dysfunctional vWF binding, were mapped to the model of human GPIb alpha. This provides the basis for relating these genetic lesions to abnormal function of the receptor. Molecular modeling of the seven tandem leucine-rich repeats within the ligand-binding region of platelet glycoprotein Ib alpha.,Whisstock JC, Shen Y, Lopez JA, Andrews RK, Berndt MC Thromb Haemost. 2002 Feb;87(2):329-33. PMID:11858495 From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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