|GPIX_HUMAN » Platelet glycoprotein IX » GP-IX; GPIX; Glycoprotein 9;|
|Hydrophobic Thickness||33.6 ± 2.0 Å|
|Tilt Angle||41 ± 1°|
|Links||UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome|
|TM Segments||148-173 (148-176)|
ECM-receptor interaction (KEGG)
Hematopoietic cell lineage (KEGG)
|UniProt annotation for GPIX_HUMAN » Platelet glycoprotein IX|
|FUNCTION: The GPIb-V-IX complex functions as the vWF receptor and mediates vWF-dependent platelet adhesion to blood vessels. The adhesion of platelets to injured vascular surfaces in the arterial circulation is a critical initiating event in hemostasis. GP-IX may provide for membrane insertion and orientation of GP-Ib. SUBUNIT: Two GP-Ib beta are disulfide-linked to one GP-Ib alpha. GP-IX is complexed with the GP-Ib heterodimer via a non covalent linkage. DISEASE: Bernard-Soulier syndrome (BSS) OMIM: A coagulation disorder characterized by a prolonged bleeding time, unusually large platelets, thrombocytopenia, and impaired prothrombin consumption. disease is caused by mutations affecting the gene represented in this entry. MISCELLANEOUS: Platelet activation apparently involves disruption of the macromolecular complex of GP-Ib with the platelet glycoprotein IX (GP-IX) and dissociation of GP-Ib from the actin- binding protein.|
|UniProt features for GPIX_HUMAN » Platelet glycoprotein IX|
SIGNAL 1 16 |
CHAIN 17 177 Platelet glycoprotein IX.
DOMAIN 17 51 LRRNT.
REPEAT 60 83 LRR.
DOMAIN 85 137 LRRCT.
|Amino Acid Sequence for GPIX_HUMAN » Platelet glycoprotein IX|
|MPAWGALFLL WATAEATKDC PSPCTCRALE TMGLWVDCRG HGLTALPALP ARTRHLLLAN NSLQSVPPGA FDHLPQLQTL DVTQNPWHCD CSLTYLRLWL EDRTPEALLQ VRCASPSLAA HGPLGRLTGY QLGSCGWQLQ ASWVRPGVLW DVALVAVAAL GLALLAGLLC ATTEALD|