GPIX_HUMAN » Platelet glycoprotein IX

GPIX_HUMAN » Platelet glycoprotein IX
Magnify GPIX_HUMAN » Platelet glycoprotein IXEnlarged view of image
3D view in GLMol or JMol

gray dot

Download Coordinates

gray dot

Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
GPIX_HUMAN » Platelet glycoprotein IX » GP-IX; GPIX; Glycoprotein 9;
Hydrophobic Thickness 30.8 ± 2.8 Å
Tilt Angle 0 ± 0°
ΔGtransfer -37.4 kcal/mol
ΔGfold -17.7 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome
Topology Out
TM Segments 148-173 (148-176)
Pathways

ECM-receptor interaction (KEGG)

Hematopoietic cell lineage (KEGG)

Hemostasis (Reactome)

PDB 3rez (45-52,65-76,92-102)
OPM none
Complexes none
Interactions

GP1BA, Complex: GPIX:GP1BA, PubMed

GP1BB, Complex: GPIX:GP1BB, PubMed

GPV, Complex: GP1BA:GPIX:GP1BB:GPV, PubMed

Domains

AA: 19-50, PDBID: 3REZ, Subunit B, Seq Identity:100%, Leucine rich repeat N-terminal domain

AA: 50-86, PDBID: 3REZ, Subunit B, Seq Identity:100%, Leucine rich repeat

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