GpIX
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/08/23 11:45:29」(JST)
[Wiki en表示]
Glycoprotein Ib (GPIb), also known as CD42,[1] is a component of the GPIb-V-IX complex on platelets. The GPIb-V-IX complex binds von Willebrand factor, allowing platelet adhesion and platelet plug formation at sites of vascular injury.
It is deficient in the Bernard-Soulier syndrome. A gain-of-function mutation causes platelet-type von Willebrand's disease.[2]
Autoantibodies against Ib/IX can be produced in immune thrombocytopenic purpura.[3]
Components include GP1BA and GP1BB.
It complexes with Glycoprotein IX.
References
- ^ Bode AP, Read MS, Reddick RL (February 1999). "Activation and adherence of lyophilized human platelets on canine vessel strips in the Baumgartner perfusion chamber". J. Lab. Clin. Med. 133 (2): 200–11. doi:10.1016/S0022-2143(99)90013-6. PMID 9989772.
- ^ McPherson & Pincus: Henry's Clinical Diagnosis and Management by Laboratory Methods, 21st ed., pp. 760-2 (W.B. Saunders, 2006).
- ^ McMillan R (October 2007). "The pathogenesis of chronic immune thrombocytopenic purpura". Semin. Hematol. 44 (4 Suppl 5): S3–S11. doi:10.1053/j.seminhematol.2007.11.002. PMID 18096470.
External links
- Glycoprotein Ib at the US National Library of Medicine Medical Subject Headings (MeSH)
Proteins: coagulation
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Coagulation factors |
Primary hemostasis |
- vWF
- platelet membrane glycoproteins: Ib (A
- B
- IX)
- IIb/IIIa (IIb
- IIIa)
- VI
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Intrinsic pathway |
- HMWK/Bradykinin
- Prekallikrein/Kallikrein
- XII "Hageman"
- XI
- IX
- VIII
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Extrinsic pathway |
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Common pathway |
- X
- V
- II "(Pro)thrombin"
- I "Fibrin"
- Fibrinogen (FGA, FGG)
- XIII
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Coagulation inhibitors |
- Antithrombin (inhibits II, IX, X, XI, XII)
- Protein C (inhibits V, VIII)/Protein S (cofactor for protein C)
- Protein Z (inhibits X)
- ZPI (inhibits X, XI)
- TFPI (inhibits III)
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Thrombolysis/fibrinolysis |
- Plasmin
- tPA/urokinase
- PAI-1/2
- α2-AP
- α2-macroglobulin
- TAFI
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Index of cells from bone marrow
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Description |
- Immune system
- Cells
- Physiology
- coagulation
- proteins
- granule contents
- colony-stimulating
- heme and porphyrin
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Disease |
- Red blood cell
- Monocyte and granulocyte
- Neoplasms and cancer
- Histiocytosis
- Symptoms and signs
- Blood tests
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Treatment |
- Transfusion
- Drugs
- thrombosis
- bleeding
- other
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UpToDate Contents
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English Journal
- Immunodiagnosis of platelet activation in immune thrombocytopenia through scFv antibodies cognate to activated IIb3 integrins.
- Bhoria P1, Varma N2, Malhotra P1, Varma S1, Luthra-Guptasarma M3.
- mAbs.MAbs.2015 Nov 2;7(6):1212-20. doi: 10.1080/19420862.2015.1075681. Epub 2015 Aug 24.
- Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by low platelet count and presence of IgG autoantibodies to platelet surface glycoproteins, such as α IIbβ3 and GPIb/IX. Our previous work has shown that platelets in ITP patients exist in an activated state. Two differ
- PMID 26301697
- Imaging the dynamic platelet-neutrophil response in sterile liver injury and repair in mice.
- Slaba I1,2, Wang J3, Kolaczkowska E3,4, McDonald B1,2,3, Lee WY2,3, Kubes P1,2,3.
- Hepatology (Baltimore, Md.).Hepatology.2015 Nov;62(5):1593-605. doi: 10.1002/hep.28003. Epub 2015 Sep 28.
- Although platelets have been extensively studied in hemostasis and inflammation, their role is not well understood in sterile liver injury and repair. Using a thermally induced focal liver injury and repair model and multichannel spinning disk confocal microscopy allowed visualization of the dynamic
- PMID 26202541
- Effect of 22q11.2 deletion on bleeding and transfusion utilization in children with congenital heart disease undergoing cardiac surgery.
- Brenner MK1, Clarke S2, Mahnke DK3, Simpson P4, Bercovitz RS5, Tomita-Mitchell A3, Mitchell ME3,6, Newman DK1,7.
- Pediatric research.Pediatr Res.2015 Oct 22. doi: 10.1038/pr.2015.216. [Epub ahead of print]
- BACKGROUND: Post-surgical bleeding causes significant morbidity and mortality in children undergoing surgery for congenital heart defects (CHD). 22q11.2 deletion syndrome (DS) is the second most common genetic risk factor for CHD. The deleted segment of chromosome 22q11.2 encompasses the gene encodi
- PMID 26492284
Japanese Journal
- GPIBとRS-232-CをPythonライブラリでサクッ! ラズパイ・コントロール! オシロ&マルチメータ自動計測システム (特設 ラズベリー・パイ実験室)
- 血小板のターンオーバーと寿命 (特集 血小板の誕生から死までの"ライフストーリー")
- von Willebrand 因子の機能と構造―A1 ドメインとGPIbαの力学的相互作用について―
Related Links
- GPIB通信の概要と用語解説、プログラミングに必要な基礎知識をわかりやすく解説します。 ... バス構造のインターフェイスを採用しており、ピギーバック方式のコネクタで機器間の接続・構成が容易です。
- 30年以上にわたって GPIB のトップメーカーである NI にお任せください。NI の GPIB ソフトウェア/ハードウェアを使用すると、性能の向上、高い信頼性、生産性の向上を実現できます。
Related Pictures
★リンクテーブル★
[★]
- 関
- CD
発現細胞
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
ファミリー
[★]
- 同
- GPIb
- 関
- GpIb, GpIX, GpV
臨床関連
[★]
血小板糖タンパク質GPIb/IX複合体
[★]
- 英
- platelet glycoprotein GPIb-IX complex
[★]
- 関
- glycosylphosphatidylinositol
[★]
- 関
- glycoprotein