Jove
Visualize
联系我们
JoVE
x logofacebook logolinkedin logoyoutube logo
关于 JoVE
概览领导团队博客JoVE 帮助中心
作者
出版流程编辑委员会范围与政策同行评审常见问题投稿
图书馆员
用户评价订阅访问资源图书馆顾问委员会常见问题
研究
JoVE JournalMethods CollectionsJoVE Encyclopedia of Experiments存档
教育
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab Manual教师资源中心教师网站
使用条款与条件
隐私政策
政策

相关概念视频

Formation of the Platelet Plug01:22

Formation of the Platelet Plug

8.6K
The platelet phase, the second stage of hemostasis, commences around 15-20 seconds after an injury. It follows and overlaps with the vascular phase, during which blood vessels constrict to minimize blood loss.
As the injured blood vessel contracts, endothelial cells undergo contraction, revealing collagen fibers in the basement membrane and underlying connective tissue. Furthermore, the plasma membrane of endothelial cells becomes adhesive, preparing the site for platelet adhesion. Platelets...
8.6K
Structure and Function of Platelets01:18

Structure and Function of Platelets

2.8K
The cell fragments known as platelets are disc-shaped, with an average diameter of about 3 μm and a thickness of roughly 1 μm. They play a crucial role in the body's vascular clotting system, which also involves plasma proteins, blood cells, and blood vessel tissues.
Platelets are continually replenished, circulating in the bloodstream for 9-12 days before being removed by phagocytes, primarily in the spleen. A microliter of circulating blood contains between 150,000 and 450,000...
2.8K
Anticoagulant Drugs: Low-Molecular-Weight Heparins01:30

Anticoagulant Drugs: Low-Molecular-Weight Heparins

1.6K
Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
1.6K
Disorders of Hemostasis01:24

Disorders of Hemostasis

2.0K
Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
2.0K
Antiplatelet Drugs: Prostaglandin Synthesis, P2Y12 and Glycoprotein IIb/IIIa Inhibitors01:20

Antiplatelet Drugs: Prostaglandin Synthesis, P2Y12 and Glycoprotein IIb/IIIa Inhibitors

1.1K
Antiplatelet drugs emerge as frontline defenders against the insidious threat of thromboembolic diseases, where abnormal clots obstruct vital blood vessels. These drugs stand as bulwarks, inhibiting platelet aggregation and clot formation, thereby mitigating the risk of life-threatening conditions like myocardial infarction, coronary artery disease, and thrombotic strokes.
Prostaglandin synthesis inhibitors, exemplified by the widely known aspirin, wield their power by irreversibly acetylating...
1.1K
Extrinsic and Intrinsic Pathways of Hemostasis01:20

Extrinsic and Intrinsic Pathways of Hemostasis

11.9K
Blood clotting or coagulation involves extrinsic and intrinsic pathways, which ultimately merge into the common pathway, forming a fibrin clot.
The Extrinsic Pathway
The extrinsic pathway of coagulation is typically initiated by tissue damage that exposes blood to tissue factor (TF), a protein released by the damaged tissue cells outside the blood vessels—this interaction with TF triggers biochemical reactions involving specific clotting factors. The key player here is Factor VII, which...
11.9K

您也可能阅读

相关文章

通过共同作者、期刊和引用图与本文相关的文章。

排序
Same author

Navigating the Hemostatic Balance: Anticoagulation and Antiplatelet Therapy in Patients with Thrombocytopenia.

Journal of clinical medicine·2026
Same author

Characterization of Novel Variants in <i>P2YRY12, GP6</i> and <i>TBXAS1</i> in Patients with Lifelong History of Bleeding.

Biomolecules·2025
Same author

Optimizing Surgical Approaches for Patients with Inherited Factor VII Deficiency.

Thrombosis and haemostasis·2025
Same author

Immune thrombocytopenia triggered by dostarlimab in a patient with endometrial carcinoma: first reported case.

Platelets·2025
Same author

Insights into the clinical, platelet and genetic landscape of inherited thrombocytopenia with malignancy risk.

British journal of haematology·2025
Same author

Diagnosis of Inherited Platelet Disorders: Clinical Evaluation and Functional and Molecular Assays.

Biomolecules·2025

相关实验视频

Updated: Jan 10, 2026

Comprehensive Analysis of Procoagulant Platelets Exhibiting Features of Necrosis, Apoptosis and Platelet Activation
04:37

Comprehensive Analysis of Procoagulant Platelets Exhibiting Features of Necrosis, Apoptosis and Platelet Activation

Published on: May 23, 2025

983

遗传性血小板功能障碍的分子病变.

Agustín Rodríguez-Alén1, Antonio Moscardó2, José M Bastida3

  • 1Servicio de Hematología, Hospital Universitario de Toledo, 45007 Toledo, Spain.

Biomolecules
|November 27, 2025
PubMed
概括

遗传性血小板功能障碍 (IPFD) 涉及导致血小板功能异常的遗传缺陷,导致出血. 基因测序方面的进步改善了诊断,并为这些疾病揭示了新的治疗点.

关键词:
格兰兹曼的血栓硬化症.赫尔曼斯基普德拉克综合征是什么遗传性血小板信号传递缺陷遗传性血小板功能障碍 遗传性血小板功能障碍血小板颗粒缺乏症 血小板颗粒缺乏症

更多相关视频

Procoagulant Platelet Characterization by Measuring Phosphatidylserine Exposure and Microvesicle Release from Human Purified Platelets
05:49

Procoagulant Platelet Characterization by Measuring Phosphatidylserine Exposure and Microvesicle Release from Human Purified Platelets

Published on: November 29, 2024

1.1K
Microfluidics in Assessing Platelet Function
06:47

Microfluidics in Assessing Platelet Function

Published on: November 8, 2024

1.5K

相关实验视频

Last Updated: Jan 10, 2026

Comprehensive Analysis of Procoagulant Platelets Exhibiting Features of Necrosis, Apoptosis and Platelet Activation
04:37

Comprehensive Analysis of Procoagulant Platelets Exhibiting Features of Necrosis, Apoptosis and Platelet Activation

Published on: May 23, 2025

983
Procoagulant Platelet Characterization by Measuring Phosphatidylserine Exposure and Microvesicle Release from Human Purified Platelets
05:49

Procoagulant Platelet Characterization by Measuring Phosphatidylserine Exposure and Microvesicle Release from Human Purified Platelets

Published on: November 29, 2024

1.1K
Microfluidics in Assessing Platelet Function
06:47

Microfluidics in Assessing Platelet Function

Published on: November 8, 2024

1.5K

科学领域:

  • 血液学 血液学 血液学
  • 分子遗传学 分子遗传学
  • 人体生理学 人体生理学

背景情况:

  • 遗传性血小板功能障碍 (IPFD) 是一种遗传性疾病,血小板数量正常,但功能受损.
  • 格兰兹曼血栓塞,一个关键的IPFD,源于对αIIbβ3整合素复合物的基因突变,导致严重出血.
  • IPFD的范围包括血小板受体,信号蛋白和颗粒生物发生的缺陷.

研究的目的:

  • 审查IPFDs的分子基础,临床特征和管理.
  • 突出先进的测序技术对IPFD诊断和理解的影响.
  • 讨论新兴的治疗策略及其对血小板生物学的影响.

主要方法:

  • 对IPFDs的当前文献的审查.
  • 对遗传变异的分析及其与临床表型的相关性.
  • 检查诊断和治疗方面的进展.

主要成果:

  • 分子遗传学已经确定了许多与IPFD相关的基因,这些基因超出了Glanzmann的血栓形成障碍.
  • 高通量测序显著提高了诊断准确性和基因型-表型相关性.
  • 目前的治疗包括抗纤维解药,德斯莫普林素和血小板输血,基因疗法和双特异性抗体是新兴的选择.

结论:

  • 了解IPFD分子病原体对于准确的诊断和有效的治疗至关重要.
  • 先进的遗传工具正在彻底改变对血小板功能障碍的研究和管理.
  • 未来的研究方向包括基因疗法和基于抗体的新疗法,以改善患者的治疗结果.