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

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Fibril-associated Collagen01:11

Fibril-associated Collagen

Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
Fibrous Proteins00:55

Fibrous Proteins

Fibrous proteins are either long and narrow proteins or assemble to form long and thin structures. They contain repetitive units and usually consist of either alpha helices or beta sheets and, in rare cases, a mix of both. The amino acids in the primary structure often consist of repeating amino acid sequences. The role of fibrous proteins is primarily structural. Many are located in the extracellular matrix and are present in connective tissues to impart strength and joint mobility. They are...
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...
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...

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

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in children ≥2 years with cystic fibrosis: 96-week interim results from a phase 3 open-label extension study.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2026
Same author

Post-infectious bronchiolitis obliterans in children: pathogenesis, treatment strategies, and future directions.

Current opinion in pediatrics·2026
Same author

Automated Ultrashort Echo Time (UTE) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy.

Journal of magnetic resonance imaging : JMRI·2026
Same author

Real-world impact of elexacaftor/tezacaftor/ivacaftor on health-related quality of life in adults with cystic fibrosis: A 12-month multicenter cohort study.

Respiratory medicine·2026
Same author

The Impact of Nutritional Status on Lung Function Trajectories in Pediatric Patients With Primary Ciliary Dyskinesia.

Pediatric pulmonology·2026
Same author

Treatment with elexacaftor/tezacaftor/ivacaftor does not alter SpiroNose-derived electronic breath profiles in children with cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2026

相关实验视频

Updated: Jul 19, 2026

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix
10:21

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

囊性纤维化症是什么

Felix Ratjen1, Gerd Döring

  • 1Department of Paediatrics, University of Essen, Essen, Germany. f.ratjen@uni-essen.de

Lancet (London, England)
|February 28, 2003
PubMed
概括

囊性纤维化是一种常见的遗传性疾病,涉及有缺陷的CFTR基因. 了解和症状治疗的进步增加了患者的预期寿命,尽管基因疗法仍然是一个挑战.

科学领域:

  • 遗传学 是一个遗传学.
  • 肺部病理学 肺部病理学
  • 生物医学研究生物医学研究

背景情况:

  • 囊性纤维化是一种普遍的自体相衰退性疾病.
  • 囊性纤维化跨膜导电性调节器 (CFTR) 基因的发现使人们对其病理生理学和诊断的理解得到了提高.
  • 尽管取得了进展,但疾病的复杂性和基因替代疗法的挑战仍然存在.

研究的目的:

  • 要总结最近在了解囊性纤维化方面的进展.
  • 审查目前的囊性纤维化治疗策略,重点关注肺部疾病.
  • 突出囊性纤维化症的复杂性和治疗开发中的持续挑战.

主要方法:

  • 关于囊性纤维化最近研究的文献综述.
  • 分析诊断工具的进步和了解CFTR基因功能.
  • 对肺部表现的当前和新兴治疗策略的评估.

主要成果:

  • 对了解囊性纤维化病理生理学和诊断的显著改进.
  • 由于加强了症状治疗策略,增加了预期寿命.
  • 基因替代疗法在有效的细胞向方面面临障碍.

结论:

更多相关视频

Microfluidic Dry-spinning and Characterization of Regenerated Silk Fibroin Fibers
08:28

Microfluidic Dry-spinning and Characterization of Regenerated Silk Fibroin Fibers

Published on: September 4, 2017

Nanofibrillar Basement Membrane Mimic Made of Recombinant Functionalized Spider Silk in Custom-Made Tissue Culture Inserts
06:17

Nanofibrillar Basement Membrane Mimic Made of Recombinant Functionalized Spider Silk in Custom-Made Tissue Culture Inserts

Published on: November 1, 2024

相关实验视频

Last Updated: Jul 19, 2026

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix
10:21

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

Microfluidic Dry-spinning and Characterization of Regenerated Silk Fibroin Fibers
08:28

Microfluidic Dry-spinning and Characterization of Regenerated Silk Fibroin Fibers

Published on: September 4, 2017

Nanofibrillar Basement Membrane Mimic Made of Recombinant Functionalized Spider Silk in Custom-Made Tissue Culture Inserts
06:17

Nanofibrillar Basement Membrane Mimic Made of Recombinant Functionalized Spider Silk in Custom-Made Tissue Culture Inserts

Published on: November 1, 2024

  • 囊性纤维化研究的进步改善了患者的治疗结果.
  • 肺部疾病仍然是致病率和死亡率的主要原因.
  • 为了有效的基因疗法和综合管理,需要进一步的研究.