Platelet glycoprotein IX

NamePlatelet glycoprotein IX
Synonyms
  • Glycoprotein 9
  • GP-IX
Gene NameGP9
OrganismHuman
Amino acid sequence
>lcl|BSEQ0016233|Platelet glycoprotein IX
MPAWGALFLLWATAEATKDCPSPCTCRALETMGLWVDCRGHGLTALPALPARTRHLLLAN
NSLQSVPPGAFDHLPQLQTLDVTQNPWHCDCSLTYLRLWLEDRTPEALLQVRCASPSLAA
HGPLGRLTGYQLGSCGWQLQASWVRPGVLWDVALVAVAALGLALLAGLLCATTEALD
Number of residues177
Molecular Weight19045.87
Theoretical pI6.32
GO Classification
Processes
  • cell adhesion
  • blood coagulation
  • platelet activation
  • blood coagulation, intrinsic pathway
Components
  • plasma membrane
  • integral component of plasma membrane
General FunctionNot Available
Specific FunctionThe 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.
Pfam Domain Function
Transmembrane Regions148-168
GenBank Protein ID2160046
UniProtKB IDP14770
UniProtKB Entry NameGPIX_HUMAN
Cellular LocationMembrane
Gene sequence
>lcl|BSEQ0016234|Platelet glycoprotein IX (GP9)
ATGCCTGCCTGGGGAGCCCTGTTCCTGCTCTGGGCCACAGCAGAGGCCACCAAGGACTGC
CCCAGCCCATGTACCTGCCGCGCCCTGGAAACCATGGGGCTGTGGGTGGACTGCAGGGGC
CACGGACTCACGGCCCTGCCTGCCCTGCCGGCCCGCACCCGCCACCTTCTGCTGGCCAAC
AACAGCCTTCAGTCCGTGCCCCCGGGAGCCTTTGACCACCTGCCCCAGCTGCAGACCCTC
GATGTGACGCAGAACCCCTGGCACTGTGACTGCAGCCTCACCTATCTGCGCCTCTGGCTG
GAGGACCGCACGCCCGAGGCCCTGCTGCAGGTCCGCTGTGCCAGCCCCAGCCTCGCTGCC
CATGGCCCGCTGGGCCGGCTGACAGGCTACCAGCTGGGCAGCTGTGGCTGGCAGCTGCAG
GCGTCCTGGGTGCGCCCGGGGGTCTTGTGGGACGTGGCGCTGGTCGCCGTGGCCGCGCTG
GGCCTGGCTCTTCTGGCTGGCCTGCTGTGTGCCACCACAGAGGCCCTGGATTGA
GenBank Gene IDX52997
GeneCard IDNot Available
GenAtlas IDGP9
HGNC IDHGNC:4444
Chromosome Location3
Locus3q21
References
  1. Hickey MJ, Deaven LL, Roth GJ: Human platelet glycoprotein IX. Characterization of cDNA and localization of the gene to chromosome 3. FEBS Lett. 1990 Nov 12;274(1-2):189-92. 2253772
  2. Hickey MJ, Roth GJ: Characterization of the gene encoding human platelet glycoprotein IX. J Biol Chem. 1993 Feb 15;268(5):3438-43. 8429020
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  4. Hickey MJ, Williams SA, Roth GJ: Human platelet glycoprotein IX: an adhesive prototype of leucine-rich glycoproteins with flank-center-flank structures. Proc Natl Acad Sci U S A. 1989 Sep;86(17):6773-7. 2771955
  5. Hayashi T, Suzuki K, Yahagi A, Akiba J, Tajima K, Satoh S, Sasaki H: Corrected DNA sequence of the platelet glycoprotein IX gene. Thromb Haemost. 1997 May;77(5):1034-5. 9184424
  6. Roth GJ, Ozols J, Nugent DJ, Williams SA: Isolation and characterization of human platelet glycoprotein IX. Biochem Biophys Res Commun. 1988 Oct 31;156(2):931-9. 3056407
  7. Gevaert K, Goethals M, Martens L, Van Damme J, Staes A, Thomas GR, Vandekerckhove J: Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides. Nat Biotechnol. 2003 May;21(5):566-9. Epub 2003 Mar 31. 12665801
  8. Wright SD, Michaelides K, Johnson DJ, West NC, Tuddenham EG: Double heterozygosity for mutations in the platelet glycoprotein IX gene in three siblings with Bernard-Soulier syndrome. Blood. 1993 May 1;81(9):2339-47. 8481514
  9. Noris P, Simsek S, Stibbe J, von dem Borne AE: A phenylalanine-55 to serine amino-acid substitution in the human glycoprotein IX leucine-rich repeat is associated with Bernard-Soulier syndrome. Br J Haematol. 1997 May;97(2):312-20. 9163595
  10. Noris P, Arbustini E, Spedini P, Belletti S, Balduini CL: A new variant of Bernard-Soulier syndrome characterized by dysfunctional glycoprotein (GP) Ib and severely reduced amounts of GPIX and GPV. Br J Haematol. 1998 Dec;103(4):1004-13. 9886312
  11. Kunishima S, Tomiyama Y, Honda S, Kurata Y, Kamiya T, Ozawa K, Saito H: Cys97-->Tyr mutation in the glycoprotein IX gene associated with Bernard-Soulier syndrome. Br J Haematol. 1999 Dec;107(3):539-45. 10583255
  12. Rivera CE, Villagra J, Riordan M, Williams S, Lindstrom KJ, Rick ME: Identification of a new mutation in platelet glycoprotein IX (GPIX) in a patient with Bernard-Soulier syndrome. Br J Haematol. 2001 Jan;112(1):105-8. 11167791
  13. Wang Z, Shi J, Han Y: [A novel point mutation in the transmembrane domain of platelet glycoprotein IX gene identified in a Bernard-Soulier syndrome patient]. Zhonghua Xue Ye Xue Za Zhi. 2001 Sep;22(9):464-6. 11758225
  14. Lanza F, De La Salle C, Baas MJ, Schwartz A, Boval B, Cazenave JP, Caen JP: A Leu7Pro mutation in the signal peptide of platelet glycoprotein (GP)IX in a case of Bernard-Soulier syndrome abolishes surface expression of the GPIb-V-IX complex. Br J Haematol. 2002 Jul;118(1):260-6. 12100158