CD42b

同

Glycoprotein Iba

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/09 09:38:03」(JST)

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Glycoprotein Ib (platelet), alpha polypeptide
PDB rendering based on 1gwb.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1GWB, 1K13, 1M0Z, 1M10, 1OOK, 1P8V, 1P9A, 1QYY, 1SQ0, 1U0N, 2BP3, 3P72, 3PMH, 4C2A, 4C2B, 4CH2, 4CH8, 4MGX
Identifiers
Symbols	GP1BA ; BDPLT1; BDPLT3; BSS; CD42B; CD42b-alpha; DBPLT3; GP1B; GPIbA; VWDP
External IDs	OMIM: 606672 MGI: 1333744 HomoloGene: 143 GeneCards: GP1BA Gene
Gene ontology Molecular function • protein binding • thrombin receptor activity Cellular component • plasma membrane • integral component of plasma membrane • membrane • anchored component of external side of plasma membrane Biological process • cell morphogenesis • cell adhesion • cell surface receptor signaling pathway • blood coagulation • blood coagulation, intrinsic pathway • platelet activation • regulation of blood coagulation • fibrinolysis • thrombin receptor signaling pathway Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	2811	14723
Ensembl	ENSG00000185245	ENSMUSG00000050675
UniProt	P07359	O35930
RefSeq (mRNA)	NM_000173	NM_010326
RefSeq (protein)	NP_000164	NP_034456
Location (UCSC)	Chr 17: 4.84 – 4.84 Mb	Chr 11: 70.64 – 70.64 Mb
PubMed search	[1]	[2]

Platelet glycoprotein Ib alpha chain also known as glycoprotein Ib (platelet), alpha polypeptide or CD42b (Cluster of Differentiation 42b), is a protein that in humans is encoded by the GP1BA gene.

Function

Glycoprotein Ib (GP Ib) is a platelet surface membrane glycoprotein composed of a heterodimer, an alpha chain and a beta chain, that are linked by disulfide bonds. The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent association of the alpha and beta subunits with platelet glycoprotein IX and platelet glycoprotein V. The binding of the GP Ib-IX-V complex to VWF facilitates initial platelet adhesion to vascular subendothelium after vascular injury, and also initiates signaling events within the platelet that lead to enhanced platelet activation, thrombosis, and hemostasis. This gene encodes the alpha subunit. Several polymorphisms and mutations have been described in this gene, some of which are the cause of Bernard-Soulier syndromes and platelet-type von Willebrand disease.

Interactions

GP1BA has been shown to interact with YWHAZ and FLNB.

See also

• Cluster of differentiation

References

Further reading

• Kunishima S, Kamiya T, Saito H (2002). "Genetic abnormalities of Bernard-Soulier syndrome". Int. J. Hematol. 76 (4): 319–27. doi:10.1007/BF02982690. PMID 12463594. 
• Du X (2007). "Signaling and regulation of the platelet glycoprotein Ib-IX-V complex". Curr. Opin. Hematol. 14 (3): 262–9. doi:10.1097/MOH.0b013e3280dce51a. PMID 17414217. 
• Clemetson KJ (2007). "A short history of platelet glycoprotein Ib complex". Thromb. Haemost. 98 (1): 63–8. doi:10.1160/th07-05-0327. PMID 17597992. 
• López JA, Ludwig EH, McCarthy BJ (1992). "Polymorphism of human glycoprotein Ib alpha results from a variable number of tandem repeats of a 13-amino acid sequence in the mucin-like macroglycopeptide region. Structure/function implications". J. Biol. Chem. 267 (14): 10055–61. PMID 1577776. 
• Murata M, Furihata K, Ishida F, Russell SR, Ware J, Ruggeri ZM (1992). "Genetic and structural characterization of an amino acid dimorphism in glycoprotein Ib alpha involved in platelet transfusion refractoriness". Blood 79 (11): 3086–90. PMID 1586750. 
• Girma JP, Takahashi Y, Yoshioka A, Diaz J, Meyer D (1990). "Ristocetin and botrocetin involve two distinct domains of von Willebrand factor for binding to platelet membrane glycoprotein Ib". Thromb. Haemost. 64 (2): 326–32. PMID 1702906. 
• Miller JL, Lyle VA, Cunningham D (1992). "Mutation of leucine-57 to phenylalanine in a platelet glycoprotein Ib alpha leucine tandem repeat occurring in patients with an autosomal dominant variant of Bernard-Soulier disease". Blood 79 (2): 439–46. PMID 1730088. 
• Modderman PW, Admiraal LG, Sonnenberg A, von dem Borne AE (1992). "Glycoproteins V and Ib-IX form a noncovalent complex in the platelet membrane". J. Biol. Chem. 267 (1): 364–9. PMID 1730602. 
• Miller JL, Cunningham D, Lyle VA, Finch CN (1991). "Mutation in the gene encoding the alpha chain of platelet glycoprotein Ib in platelet-type von Willebrand disease". Proc. Natl. Acad. Sci. U.S.A. 88 (11): 4761–5. doi:10.1073/pnas.88.11.4761. PMC 51746. PMID 2052556. 
• Hess D, Schaller J, Rickli EE, Clemetson KJ (1991). "Identification of the disulphide bonds in human platelet glycocalicin". Eur. J. Biochem. 199 (2): 389–93. doi:10.1111/j.1432-1033.1991.tb16135.x. PMID 2070794. 
• Du X, Beutler L, Ruan C, Castaldi PA, Berndt MC (1987). "Glycoprotein Ib and glycoprotein IX are fully complexed in the intact platelet membrane". Blood 69 (5): 1524–7. PMID 2436691. 
• Andrews RK, Booth WJ, Gorman JJ, Castaldi PA, Berndt MC (1989). "Purification of botrocetin from Bothrops jararaca venom. Analysis of the botrocetin-mediated interaction between von Willebrand factor and the human platelet membrane glycoprotein Ib-IX complex". Biochemistry 28 (21): 8317–26. doi:10.1021/bi00447a009. PMID 2557900. 
• Wenger RH, Wicki AN, Kieffer N, Adolph S, Hameister H, Clemetson KJ (1989). "The 5' flanking region and chromosomal localization of the gene encoding human platelet membrane glycoprotein Ib alpha". Gene 85 (2): 517–24. doi:10.1016/0378-1119(89)90446-0. PMID 2628181. 
• Wenger RH, Kieffer N, Wicki AN, Clemetson KJ (1988). "Structure of the human blood platelet membrane glycoprotein Ib alpha gene". Biochem. Biophys. Res. Commun. 156 (1): 389–95. doi:10.1016/S0006-291X(88)80853-2. PMID 2845978. 
• Wicki AN, Clemetson KJ (1985). "Structure and function of platelet membrane glycoproteins Ib and V. Effects of leukocyte elastase and other proteases on platelets response to von Willebrand factor and thrombin". Eur. J. Biochem. 153 (1): 1–11. doi:10.1111/j.1432-1033.1985.tb09259.x. PMID 2933256. 
• Adelman B, Michelson AD, Greenberg J, Handin RI (1986). "Proteolysis of platelet glycoprotein Ib by plasmin is facilitated by plasmin lysine-binding regions". Blood 68 (6): 1280–4. PMID 2946332. 
• Lopez JA, Chung DW, Fujikawa K, Hagen FS, Papayannopoulou T, Roth GJ (1987). "Cloning of the alpha chain of human platelet glycoprotein Ib: a transmembrane protein with homology to leucine-rich alpha 2-glycoprotein". Proc. Natl. Acad. Sci. U.S.A. 84 (16): 5615–9. doi:10.1073/pnas.84.16.5615. PMC 298913. PMID 3303030. 
• Lopez JA, Chung DW, Fujikawa K, Hagen FS, Davie EW, Roth GJ (1988). "The alpha and beta chains of human platelet glycoprotein Ib are both transmembrane proteins containing a leucine-rich amino acid sequence". Proc. Natl. Acad. Sci. U.S.A. 85 (7): 2135–9. doi:10.1073/pnas.85.7.2135. PMC 279943. PMID 3353370. 
• Titani K, Takio K, Handa M, Ruggeri ZM (1987). "Amino acid sequence of the von Willebrand factor-binding domain of platelet membrane glycoprotein Ib". Proc. Natl. Acad. Sci. U.S.A. 84 (16): 5610–4. doi:10.1073/pnas.84.16.5610. PMC 298912. PMID 3497398. 
• Harmon JT, Jamieson GA (1986). "The glycocalicin portion of platelet glycoprotein Ib expresses both high and moderate affinity receptor sites for thrombin. A soluble radioreceptor assay for the interaction of thrombin with platelets". J. Biol. Chem. 261 (28): 13224–9. PMID 3759960. 

External links

• GP1BA protein, human at the US National Library of Medicine Medical Subject Headings (MeSH)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

English Journal

• Storage of apheresis platelet concentrates after manual replacement of >95% of plasma with PAS 5.

• Morrison A1, McMillan L, Radwanski K, Blatchford O, Min K, Petrik J.
• Vox sanguinis.Vox Sang.2014 Oct;107(3):247-53. doi: 10.1111/vox.12157. Epub 2014 May 7.
• BACKGROUND AND OBJECTIVES: Recently, a glucose- and bicarbonate-containing additive solution termed PAS 5 demonstrated acceptable 7-day platelet storage after >95% plasma replacement with PAS on the day of collection (Day 0). In this study, we examined platelet storage in >95% PAS 5 after manu
• PMID 24801569

• Reduced platelet hyperreactivity and platelet-monocyte aggregation in HIV-infected individuals receiving a raltegravir-based regimen.

• Tunjungputri RN1, Van Der Ven AJ, Schonsberg A, Mathan TS, Koopmans P, Roest M, Fijnheer R, Groot PG, de Mast Q.
• AIDS (London, England).AIDS.2014 Sep 10;28(14):2091-6. doi: 10.1097/QAD.0000000000000415.
• OBJECTIVE: Platelets are key cells in atherosclerosis and acute cardiovascular events. Platelet hyperreactivity and increased platelet-monocyte aggregation (PMA) are found in HIV-infected patients and may contribute to the excess cardiovascular risk. The integrase inhibitor raltegravir (RAL) has bee
• PMID 25265076

• Successful treatment with oseltamivir phosphate in a patient with chronic immune thrombocytopenia positive for anti-GPIb/IX autoantibody.

• Shao L1, Wu Y, Zhou H, Qin P, Ni H, Peng J, Hou M.
• Platelets.Platelets.2014 Aug 28:1-3. [Epub ahead of print]
• Abstract The management of chronic immune thrombocytopenia (ITP) remains to be a challenge. Oseltamivir phosphate is a sialidase inhibitor agent used to treat influenza in the conventional sense. At present, we demonstrate for the first time that an adult chronic ITP patient with anti-GP Ib/IX autoa
• PMID 25166956

Japanese Journal

• ヒト巨核球系細胞の増殖,分化に果たすフィブロネクチンの役割

• 深澤 元晴,フカザワ モトハル,FUKAZAWA Motoharu
• 千葉医学雑誌 73(2), 87-96, 1997-04-01
• … FNは分化の指標となるCD34, CD41a (GPHb/IIIa), CD42b (GP I b)に影響を与えなかった。 …
• NAID 110004650930

• Effects of yperthermia on Human Platelets. Flow cytometric analysis on the formation of platelet-derived microparticles and the expression of CD62 antigen on the platelet surface.:Flow cytometric analysis on the formation of platelet-derived microparticles and the expression of CD62 antigen on the platelet surface

• 武 仁,久保田 一雄,田村 耕成,倉林 均,田村 遵一,白倉 卓夫,森 真由美
• 日本温泉気候物理医学会雑誌 58(4), 213-217, 1995
• … Then 2μl of each sample was incubated with FITC or PE-conjugated anti-human CD42b or CD62 antibodies, and assayed for MP and CD62 by flow cytometry. …
• NAID 130002042539

Related Links

• 抗CD42b抗体 | サイトメトリードットコム/ベックマン・コールター

CD42b抗原（GPⅠbα）は135kDaの膜糖タンパクで、CD42c（GPⅠbβ）とジスルフィド結合して160kDaの膜タンパクを形成します。このヘテロダイマー（GPⅠb）は、CD42a（GPⅨ）及びCD42d（GPⅤ）と非共有結合的に複合体を形成します。

• CD42b 抗体, ヒト - Miltenyi Biotec - MACSのミルテニーバイオテク

クローン REA185 は、145 kDa の1回膜貫通型のタイプⅠ タンパク質 CD42b を認識します。CD42b の発現は、血小板と巨核球に認められます。CD42b (GP Ib, α 鎖) は CD42c (GP Ib, β鎖) と共にヘテロ2量体である glycoprotein Ib (GP Ib) を ...

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★リンクテーブル★

リンク元	「ベルナール・スリエ症候群」
関連記事	「CD42」「CD4」

「ベルナール・スリエ症候群」

　　[★]

英

Bernard-Soulier syndrome, BSS

同

Bernard-Soulier症候群

関

血小板、出血性疾患

概念

• von Willebrand病と同様に、皮膚・粘膜下出血、リストセチン凝集欠如。ADP凝集やコラーゲン凝集は正常であるが、vWF活性が正常、血小板軽度減少する点が異なる。

血液検査結果はGlanzmann's thrombastheniaに似る　→　出血性疾患

病因

• GpIb/GpIX/GpV複合体の欠如・減少。　←　GpIbα、GpIbβ、GpIXの遺伝子変異&発現の低下による

これらは血小板上で複合体を形成し、コラーゲンに結合したフォンウイルブランド因子と結合する。これらが失われることで(特に流れの速い血管部位で)血小板の凝集が起こらなくなる。これにより出血時間が延長する。

疫学

• 常染色体劣性遺伝

症状

• 出血傾向は生後より出現
• 皮膚・粘膜下出血、鼻出血、歯肉出血、性器出血、血尿、消化管出血

検査

• 末梢血血小板：巨大血小板の出現(直径15-20μm)
• 出血時間：延長
• 骨髄巨核球数：正常。
• 血小板数：正常ないし減少。
• 血小板機能検査

• リストセチン凝集、トロンビン凝集：欠如or低下
• ADP凝集：正常
• コラーゲン凝集：正常

• 血小板表面マーカーCD42b：著減

治療

• 特異的な治療法はなし。
• 新鮮血小板輸血が最も確実な止血法。

「CD42」

　　[★]

関

CD

発現細胞

• 血小板、巨核芽球

分子量

• a:23, B:135&23, c:22, d:85

機能

• binds von Willebrand factor, thrombin; essential for platelet adhesion at sites of injury

別名

• a: GPIX, b:GPIbα, c:GPIbβ, d: GPV

ファミリー

• leucine-rich repeat

「CD4」

　　[★]

同

T4, Leucine-SA, Leucine-3B

関

表面抗原分類