GP1BA_HUMAN » Platelet glycoprotein Ib alpha chain

GP1BA_HUMAN » Platelet glycoprotein Ib alpha chain
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
GP1BA_HUMAN » Platelet glycoprotein Ib alpha chain » GP-Ib alpha; GPIb-alpha; GPIbA; Glycoprotein Ibalpha; Antigen CD42b-alpha;
Hydrophobic Thickness 34.4 ± 1.2 Å
Tilt Angle 1 ± 2°
ΔGtransfer -27.6 kcal/mol
ΔGfold -20.8 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 507-530 (507-533)
Pathways

ECM-receptor interaction (KEGG)

Hematopoietic cell lineage (KEGG)

Hemostasis (Reactome)

PDB 1gwb (16-296), 1m0z (17-306), 1p9a (17-306), 1qyy (17-306), 3p72 (A=17-281), 1p8v (A=17-294), 4c2b (B/D/F/H=17-306), 1sq0 (B=17-304), 1m10 (B=17-306), 4c2a (B=17-306), 4mgx (B=603-611), 1u0n (D=17-281), 3pmh (G=17-306), 1ook (G=17-306), 4ch8 (P/Q/R/S=287-300), 4ch2 (P/Q=287-300), 2bp3 (S/T=598-619)
OPM none
Complexes none
Interactions

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

GPIX, Complex: GPIX:GP1BA, PubMed

GPV, Complex: GP1BA:GPV, PubMed

ITAM, Complex: ITAM:GP1BA, PubMed

LYAM3, Complex: GP1BA:LYAM3, PubMed

TPST1, Complex: GP1BA:TPST1, PubMed

Domains

AA: 19-46, PDBID: 1GWB, Subunit B, Seq Identity:100%, Leucine rich repeat N-terminal domain

AA: 116-176, PDBID: 1GWB, Subunit B, Seq Identity:100%, Leucine rich repeat

AA: 164-222, PDBID: 1GWB, Subunit B, Seq Identity:100%, Leucine rich repeat

UniProt annotation for GP1BA_HUMAN » Platelet glycoprotein Ib alpha chain
FUNCTION: GP-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to the A1 domain of vWF, 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. Interacts with FLNB.

DISEASE: Non-arteritic anterior ischemic optic neuropathy (NAION) OMIM: An ocular disease due to ischemic injury to the optic nerve. It usually affects the optic disk and leads to visual loss and optic disk swelling of a pallid nature. Visual loss is usually sudden, or over a few days at most and is usually permanent, with some recovery possibly occurring within the first weeks or months. Patients with small disks having smaller or non- existent cups have an anatomical predisposition for non-arteritic anterior ischemic optic neuropathy. As an ischemic episode evolves, the swelling compromises circulation, with a spiral of ischemia resulting in further neuronal damage. associated with variations affecting the gene represented in this entry.

DISEASE: Bernard-Soulier syndrome (BSS) OMIM: A coagulation disorder characterized by a prolonged bleeding time, unusually large platelets, thrombocytopenia, and impaired prothrombin consumption. mutations affecting the gene represented in this entry.

DISEASE: Bernard-Soulier syndrome A2, autosomal dominant (BSSA2) OMIM: A coagulation disorder characterized by mild to moderate bleeding tendency, thrombocytopenia, and an increased mean platelet volume. Some individuals have no symptoms. Mild bleeding tendencies manifest as epistaxis, gingival bleeding, menorrhagia, easy bruising, or prolonged bleeding after dental surgery. by mutations affecting the gene represented in this entry.

DISEASE: Pseudo-von Willebrand disease (VWDP) OMIM: A bleeding disorder characterized by abnormally enhanced binding of von Willebrand factor by the platelet glycoprotein Ib (GP Ib) receptor complex. Hemostatic function is impaired due to the removal of VWF multimers from the circulation. 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.

MISCELLANEOUS: Binding sites for vWF and thrombin (the latter site with unknown function) are in the N-terminal part of the molecule.

UniProt features for GP1BA_HUMAN » Platelet glycoprotein Ib alpha chain
SIGNAL 1 16
CHAIN 17 626 Platelet glycoprotein Ib alpha chain.
CHAIN 17 ? Glycocalicin.
DOMAIN 17 47 LRRNT.
REPEAT 48 68 LRR 1.
REPEAT 72 93 LRR 2.
REPEAT 94 115 LRR 3.
REPEAT 117 137 LRR 4.
REPEAT 141 162 LRR 5.
REPEAT 165 186 LRR 6.
REPEAT 189 210 LRR 7.
DOMAIN 221 282 LRRCT.
DISULFID 20 33
DISULFID 225 264
DISULFID 227 280
DISULFID 500 500 Interchain (with C-147 in GP1BB).
DISULFID 501 501 Interchain (with C-147 in GP1BB).
Amino Acid Sequence for GP1BA_HUMAN » Platelet glycoprotein Ib alpha chain
MPLLLLLLLL PSPLHPHPIC EVSKVASHLE VNCDKRNLTA LPPDLPKDTT ILHLSENLLY TFSLATLMPY TRLTQLNLDR CELTKLQVDG TLPVLGTLDL SHNQLQSLPL LGQTLPALTV LDVSFNRLTS LPLGALRGLG ELQELYLKGN ELKTLPPGLL TPTPKLEKLS LANNNLTELP AGLLNGLENL DTLLLQENSL YTIPKGFFGS HLLPFAFLHG NPWLCNCEIL YFRRWLQDNA ENVYVWKQGV DVKAMTSNVA SVQCDNSDKF PVYKYPGKGC PTLGDEGDTD LYDYYPEEDT EGDKVRATRT VVKFPTKAHT TPWGLFYSWS TASLDSQMPS SLHPTQESTK EQTTFPPRWT PNFTLHMESI TFSKTPKSTT EPTPSPTTSE PVPEPAPNMT TLEPTPSPTT PEPTSEPAPS PTTPEPTPIP TIATSPTILV SATSLITPKS TFLTTTKPVS LLESTKKTIP ELDQPPKLRG VLQGHLESSR NDPFLHPDFC CLLPLGFYVL GLFWLLFASV VLILLLSWVG HVKPQALDSG QGAALTTATQ TTHLELQRGR QVTVPRAWLL FLRGSLPTFR SSLFLWVRPN GRVGPLVAGR RPSALSQGRG QDLLSTVSIR YSGHSL