Jove
Visualize
お問い合わせ

関連する概念動画

Desmosomes01:05

Desmosomes

5.6K
The term desmosome derives from the Greek words "desmo" and "soma" meaning "adhesion bodies." This structure was first observed during the late 1800s and described as small, dense nodules in the epidermis. Desmosomes are button-like structures that help form an interlinked network of intermediate filaments across the cells. These junctions are  essential to hold cells together under mechanical stress and to maintain tissue integrity. Desmosomes are multi-protein...
5.6K
Intracellular Signaling Affects Focal Adhesions01:17

Intracellular Signaling Affects Focal Adhesions

2.8K
Integrins act both as extracellular input receivers and as intracellular processing activators. As their name suggests, integrins are entirely integrated into the membrane structure. Their hydrophobic membrane-spanning regions interact with the phospholipid bilayer's hydrophobic region. These membrane receptors provide extracellular attachment sites for effectors like hormones and growth factors. They activate intracellular response cascades when their effectors are bound and active.
Some...
2.8K
Tension Response at Adherens Junctions01:26

Tension Response at Adherens Junctions

2.8K
The adherens junctions that anchor cells together are multi-protein complexes that dynamically adapt to mechanical stimuli such as tensile forces and shear stress. Mechanosensory proteins in these junctions can sense such mechanical stimuli and undergo a shift in their conformation, resulting in an altered function — a process called mechanotransduction.
α-Catenin as a Mechanosensory Protein
The α-catenin of adherens junctions is an allosteric protein with three VH (vinculin...
2.8K
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

37
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
37
Anchoring Junctions01:03

Anchoring Junctions

3.9K
Anchoring junctions are multiprotein complexes that help cells connect to other cells and the extracellular matrix. Anchoring junctions are present on the lateral and basal surfaces of cells, providing strong and flexible connections. Focal adhesions are often formed due to cell interactions with the ECM substrata, which initiate signal transduction via kinase cascades and other mechanisms. Together, they provide stability and tissue integrity. There are three types of anchoring junctions:...
3.9K
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

31
Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
31
JoVE
x logofacebook logolinkedin logoyoutube logo
JoVEについて
概要リーダーシップブログJoVEヘルプセンター
著者向け
出版プロセス編集委員会範囲と方針査読よくある質問投稿
図書館員向け
推薦の声購読アクセスリソース図書館諮問委員会よくある質問
研究
JoVE JournalMethods CollectionsJoVE Encyclopedia of Experimentsアーカイブ
教育
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab Manual教員リソースセンター教員サイト
利用規約
プライバシーポリシー
ポリシー
  1. ホーム
  2. インテグリン-αvβ6/tgf-βシグナリングカスケードを伴う不全性デスマソーマルアデシオパシー
  1. ホーム
  2. インテグリン-αvβ6/tgf-βシグナリングカスケードを伴う不全性デスマソーマルアデシオパシー

関連する実験動画

Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients
09:16

Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients

Published on: February 28, 2018

7.8K

インテグリン-αVβ6/TGF-βシグナリングカスケードを伴う不全性デスマソーマルアデシオパシー

Camilla Schinner1, Lifen Xu2, Henriette Franz1

  • 1Department of Biomedicine, Section Anatomy (C. Schinner, H.F., A.Z., M.-T.W., M.R., P.H., C. Stüdle, P.I.M., V.S.), University of Basel, Switzerland.

Circulation
|October 21, 2022

PubMed で要約を見る

まとめ
この要約は機械生成です。

デスマグレーン-2の結合を阻害すると,マウスの心律異常 (ACM) が起こり,インテグリン-αVβ6が線維症の標的となる. この研究はACMの機能不全を確認し,潜在的な治療経路を特定しました.

キーワード:
心筋病デスマソーム繊維症インテグリンインターカレートディスク成長因子を変化させる

さらに関連する動画

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

10.1K
Analysis of Tubular Membrane Networks in Cardiac Myocytes from Atria and Ventricles
10:30

Analysis of Tubular Membrane Networks in Cardiac Myocytes from Atria and Ventricles

Published on: October 15, 2014

20.7K

関連する実験動画

Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients
09:16

Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients

Published on: February 28, 2018

7.8K
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

10.1K
Analysis of Tubular Membrane Networks in Cardiac Myocytes from Atria and Ventricles
10:30

Analysis of Tubular Membrane Networks in Cardiac Myocytes from Atria and Ventricles

Published on: October 15, 2014

20.7K

科学分野:

  • 心臓病科
  • 分子生物学
  • 生物化学

背景:

  • 動脈動乱性心筋病 (ACM) は,心筋細胞の喪失と繊維脂肪の置換を伴うもので,しばしばデスマソーム遺伝子変異と関連している.
  • デスマソームの欠陥がACMの病原化に寄与する正確なメカニズムは,まだ完全に理解されていません.
  • この研究では,ACMの発症と進行におけるデスマソーマ結合障害の役割を調査しています.

研究 の 目的:

  • 動脈動乱性心筋病 (ACM) の発現における欠陥性デスマソーマ粘着の関連性を調査する.
  • デスモソーム機能障害と心筋線維症と心律乱を結びつける分子メカニズムを解明する.
  • ACMの潜在的治療標的を特定する

主な方法:

  • マウスモデルでデスマゲリン-2 (DSG2) 変異 (DSG2-W2A) を設計し,デスマソーム結合を妨害した.
  • 変異性マウスにおける心臓機能と組織学的評価は,エコーカルディオグラフィー,心電図,および高度顕微鏡を用いて行われました.
  • 分析されたトランスクリプトミックのデータとACM患者のサンプルと心臓スライス培養で検証された結果

主要な成果:

  • DSG2- W2A変異は細胞間粘着を低下させ,線維症と静脈機能不全を含むマウスのACMのようなフェノタイプを誘発した.
  • トランスクリプトミア分析により,インテグリン-αVβ6とTGF-βの信号放出が心臓線維症の主要な要因であることが明らかになった.
  • インテグリン-αVβ6の抑制は,変異したマウスのプロフィブロティックマーカーと線維症を減少させた.
  • 結論:

    • デスモソーム粘着の障害はACMを引き起こすのに十分であり,機能不全の粘着仮説を支持する.
    • インテグリン-αVβ6とTGF-βのシグナル伝達経路は,ACMにおける心臓線維症の重要なメディエーターである.
    • インテグリン-αVβ6経路をターゲットにすることで,ACMにおける線維症の改善が期待されています.