GP1BB_HUMAN » Platelet glycoprotein Ib beta chain

GP1BB_HUMAN » Platelet glycoprotein Ib beta chain
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
GP1BB_HUMAN » Platelet glycoprotein Ib beta chain » GP-Ib beta; GPIb-beta; GPIbB; Antigen CD42b-beta;
Hydrophobic Thickness 34.4 ± 2.8 Å
Tilt Angle 20 ± 8°
ΔGtransfer -21.5 kcal/mol
ΔGfold -21.7 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 151-175 (150-182)
Pathways

ECM-receptor interaction (KEGG)

Hematopoietic cell lineage (KEGG)

Hemostasis (Reactome)

PDB 3rez (26-146), 3rfe (26-146)
OPM none
Complexes none
Interactions

GP1BA, Complex: GP1BA:GPIX:GP1BB:VWF, PubMed

GPIX, Complex: GPIX:GP1BB, PubMed

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

VCAM1, Complex: GP1BB:VCAM1

Domains

AA: 115-142, PDBID: 3REZ, Subunit D, Seq Identity:100%, Leucine rich repeat C-terminal domain

UniProt annotation for GP1BB_HUMAN » Platelet glycoprotein Ib beta chain
FUNCTION: Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.

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.

TISSUE SPECIFICITY: Expressed in heart and brain.

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 GP1BB_HUMAN » Platelet glycoprotein Ib beta chain
SIGNAL 1 26
CHAIN 27 206 Platelet glycoprotein Ib beta chain.
DOMAIN 27 55 LRRNT.
REPEAT 60 83 LRR.
DOMAIN 89 143 LRRCT.
DISULFID 26 32
DISULFID 30 39
DISULFID 93 118
DISULFID 95 141
DISULFID 147 147 Interchain (with C-500 or C-501 in GP1BA).
Amino Acid Sequence for GP1BB_HUMAN » Platelet glycoprotein Ib beta chain
MGSGPRGALS LLLLLLAPPS RPAAGCPAPC SCAGTLVDCG RRGLTWASLP TAFPVDTTEL VLTGNNLTAL PPGLLDALPA LRTAHLGANP WRCDCRLVPL RAWLAGRPER APYRDLRCVA PPALRGRLLP YLAEDELRAA CAPGPLCWGA LAAQLALLGL GLLHALLLVL LLCRLRRLRA RARARAAARL SLTDPLVAER AGTDES