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相关概念视频

Caspases01:24

Caspases

Caspase, a family of cysteine proteases, serve as effectors in apoptosis. The ced3 gene in C.elegans was first identified to be involved in apoptosis. This gene encodes the ced-3 caspase that is similar to the interleukin-1-beta converting enzyme or ICE in mammals. In addition to apoptosis, caspases also function in the inflammatory response. Inflammatory caspases are essential in activating pro-inflammatory cytokines that recruit immune cells and block the replication of pathogens inside cells.
The Extrinsic Apoptotic Pathway01:17

The Extrinsic Apoptotic Pathway

The extrinsic apoptotic pathway is initiated when extracellular death-inducing signals, such as specific cytokines, activate the death receptors expressed on the cell surface. The immune cells involved in this pathway are natural killer cells (NK cells) and cytotoxic T-lymphocytes. NK cells are critical in innate immune response, while cytotoxic T-lymphocytes are associated with adaptive immune response. These cells recognize specific receptors expressed on the altered cells and activate...
The Intrinsic Apoptotic Pathway01:31

The Intrinsic Apoptotic Pathway

Internal cellular stress, such as cellular injury or hypoxia, triggers intrinsic apoptosis. The B-cell lymphoma 2 (Bcl-2) family of proteins are the primary regulators of the intrinsic apoptotic pathway. For example, during DNA damage, checkpoint proteins, such as Ataxia Telangiectasia Mutated (ATM protein) and Checkpoints Factor-2 (Chk2) proteins, are activated. These proteins phosphorylate p53 which further activates pro-apoptotic proteins, such as Bax, Bak, PUMA, and Noxa, and inhibits...
Immunodeficiency Diseases01:25

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...

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相关实验视频

Updated: Jun 19, 2026

Lighting Up the Pathways to Caspase Activation Using Bimolecular Fluorescence Complementation
08:47

Lighting Up the Pathways to Caspase Activation Using Bimolecular Fluorescence Complementation

Published on: March 5, 2018

由caspase-8突变引起的淋巴细胞激活中的类缺陷导致人类免疫缺陷.

Hyung J Chun1, Lixin Zheng, Manzoor Ahmad

  • 1Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA.

Nature
|September 28, 2002
PubMed
概括

人类遗传的卡斯帕-8缺乏导致免疫系统疾病,与ALPS不同,揭示了其在激活天真淋巴细胞和维持免疫平衡中的关键产后作用.

更多相关视频

Measuring Composition of CD95 Death-Inducing Signaling Complex and Processing of Procaspase-8 in this Complex
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Measuring Composition of CD95 Death-Inducing Signaling Complex and Processing of Procaspase-8 in this Complex

Published on: August 2, 2021

Evaluation of Caspase Activation to Assess Innate Immune Cell Death
10:23

Evaluation of Caspase Activation to Assess Innate Immune Cell Death

Published on: January 20, 2023

相关实验视频

Last Updated: Jun 19, 2026

Lighting Up the Pathways to Caspase Activation Using Bimolecular Fluorescence Complementation
08:47

Lighting Up the Pathways to Caspase Activation Using Bimolecular Fluorescence Complementation

Published on: March 5, 2018

Measuring Composition of CD95 Death-Inducing Signaling Complex and Processing of Procaspase-8 in this Complex
07:17

Measuring Composition of CD95 Death-Inducing Signaling Complex and Processing of Procaspase-8 in this Complex

Published on: August 2, 2021

Evaluation of Caspase Activation to Assess Innate Immune Cell Death
10:23

Evaluation of Caspase Activation to Assess Innate Immune Cell Death

Published on: January 20, 2023

科学领域:

  • 免疫学 免疫学 免疫学
  • 分子生物学分子生物学
  • 遗传学 是一个遗传学.

背景情况:

  • 细胞亡,或编程细胞死亡,由卡斯帕斯细胞调节,对免疫恒温至关重要,防止自身免疫.
  • 淋巴细胞亡的缺陷与自身免疫淋巴增殖综合征 (ALPS) 相关,通常是由CD95,CD95连接体或caspase-10的突变引起的.
  • 在ALPS中没有发现caspase-8突变,而它的完全缺乏在小鼠中是致命的.

研究的目的:

  • 研究人类遗传性caspase-8遗传缺陷的影响.
  • 了解卡斯帕-8在淋巴细胞亡,平衡和免疫细胞激活中的作用.
  • 为了区分卡斯巴-8缺乏与ALPS.

主要方法:

  • 临床和基因分析人类同胞遗传caspase-8缺乏症.
  • 在受影响的个体中评估淋巴细胞亡和恒温.
  • 评估T淋巴细胞,B淋巴细胞和自然杀手细胞的激活.

主要成果:

  • 在人类中,同卵性酶-8缺乏导致缺陷的淋巴细胞亡和恒常.
  • 受影响的个体表现出T细胞,B细胞和NK细胞的激活受损,导致免疫缺陷.
  • 与ALPS不同,卡斯巴酶-8缺乏与正常发育相容.

结论:

  • 与ALPS相比,人体酶-8缺乏症具有明显的免疫缺陷.
  • 卡斯巴-8在活化原始淋巴细胞方面发挥着关键的产后作用.
  • 这项研究强调了caspase-8在出生后维持免疫系统功能方面以前未知的功能.