Name: desmosomes and cell anchoring
Uploaded: 2025-05-22T09:05:46.000Z
Duration: 1 min 5 s
Channel: JoVE

데스모솜(Desmosome)은 심장 근육 및 장 상피와 같은 기계적 스트레스를 받는 조직에서 더 풍부하게 발견되는 고정 연접부입니다.
데스모솜에서의 세포 접착은 데스모솜 cadherins, 즉 데스모콜린(desmocollin) 및 데스모글린(desmoglein)에 의해 촉진되며, 이는 세포 외 도메인의 이질성 상호 작용을 통해 부착됩니다.
cadherins의 세포질 도메인은 플라코글로빈(plakoglobins)과 플라코필린(plakophilins)이라고 하는 카테닌과 결합하며, 이는 세포질 쪽에 조밀한 플라크를 형성합니다.
카테닌은 중간 필라멘트와 직접 결합할 수 없으며, 플라킨 계열에 속하는 세포골격 링커 단백질인 데스모플라킨(desmoplakin)에 의해 연결되어 있습니다.
Desmoplakin의 N-말단 도메인은 카테닌과 결합하고 C-말단 플라킨 반복은 중간 필라멘트와 결합합니다.
따라서 데스모솜은 인접 세포의 중간 필라멘트를 고정하여 조직을 가로질러 세포골격 네트워크를 형성합니다.

<ol>
	<li>델바, 엠마누엘라, 다나 K. 터커, 앤드류 P. 코발치크. "데스모솜." 생물학의 콜드 스프링 하버 관점 1.2 (2009): a002543.</li>
	<li>Garrod, David 및 Martyn Chidgey. "데스모솜 구조, 구성 및 기능." Biochimica et Biophysica Acta (BBA)-Biomembranes 1778.3 (2008): 572-587.</li>
	<li>Nekrasova, Oxana 및 Kathleen J. Green. "데스모솜 어셈블리 및 역학." 세포 생물학 동향 23.11 (2013): 537-546.</li>
</ol>

부착반점

Desmosomes

부착반점이라는 용어는 "유착체"를 의미하는 그리스어 "desmo"와 "soma"에서 유래되었습니다. 이 구조는 1800년대 후반에 처음 관찰되었으며 표피의 작고 조밀한 결절로 설명되었습니다. 데스모좀은 세포 전체에 걸쳐 중간 필라멘트의 상호 연결된 네트워크를 형성하는 데 도움이 되는 단추 모양의 구조입니다. 이러한 접합은 기계적 스트레스 하에서 세포를 함께 유지하고 조직 무결성을 유지하는 데 필수적입니다. 부착반점은 다양한 어댑터 단백질을 통해 중간 필라멘트에 연결된 부착반점 카드헤린으로 구성된 다중 단백질 복합체입니다.

부착반점 카드헤린

카드헤린은 부착반점의 세포외 핵심 영역을 형성하고 인접한 세포의 카드헤린과 결합합니다. 데스모좀에는 데스모글레인과 데스모콜린이라는 두 가지 유형의 카드헤린이 있습니다. 이 막횡단 당단백질은 4개의 카드헤린 반복부와 이 단백질을 세포막에 고정시키는 1개의 세포외 고정 도메인으로 구성된 큰 세포외 부분을 보유합니다. 데스모글레인과 데스모콜린의 이종이합체 쌍은 동일한 세포에서의 시스 상호작용과 인접한 세포 간의 트랜스 상호작용에 의해 클러스터를 형성합니다. 이의 세포질 도메인은 대체 접합으로 인해 서로 다른 이소형 사이에서 길이가 다를 수 있습니다. 예를 들어 인간에서는 4개의 데스모글레인 이소형과 3개의 데스모콜린 이소형이 발견됩니다.

부착반점의 어댑터 단백질

부착반점 카드헤린의 세포질 도메인은 플라코글로빈 및 플라코필린이라고 불리는 아르마딜로 단백질 계열과 결합하여 치밀한 플라크를 형성합니다. 이 단백질은 데스모플라킨과 같은 다양한 링커 단백질의 도킹 부위로 기능하는 특징적인 팔 반복을 가지고 있습니다. 데스모플라킨은 부착반점 복합체에서 가장 풍부한 단백질입니다. 카르복시 말단에는 중간 필라멘트를 결합하는 플라킨 반복이 포함되어 있어 부착반점 플라크와 세포골격 사이의 연결을 형성합니다.

부착반점 기능 장애의 질병

부착반점 기능 장애는 일반적으로 심장과 피부의 장애로 나타납니다. 조직은 일반적으로 높은 수준의 기계적 긴장을 겪고 있습니다. 예를 들어, 데스모글레인 유전자의 돌연변이는 표피 비후와 부정맥성 우심실 심근병증(ARVC)을 유발할 수 있습니다. 또한, 부착반점 카드헤린을 표적으로 하는 자가항체는 자가면역질환인 심상성 천포창을 유발하는데, 이 질환에서는 환자가 약한 세포-세포 접착으로 인해 표피 수포를 나타냅니다.

데스모솜이라는 용어는 그리스어 "desmo"와 "soma"에서 파생된 것으로 "접착체"를 의미합니다. 이 구조는 처음 관찰되었습니다 ...

Education

교육

JoVE Core

Cell Biology

세포생물학

Unit 1

제1단원

Cell-Cell Interactions

세포 간 상호작용

KEY TERMS AND CONCEPTS

주요 용어 및 개념

desmosomes and cell anchoring

Desmosomes are cell junctions that anchor cells together. They are button-like structures that create an interlinked network of intermediate filaments across cells. This strong link helps tissues resist mechanical stress and maintain tissue integrity.

Desmosomes are built from multiple proteins. Their core includes desmosomal cadherins, which are adhesion proteins that bind to cadherins on neighboring cells. The two desmosomal cadherins are desmogleins and desmocollins. These transmembrane glycoproteins have a large extracellular region with four cadherin repeats and one anchor domain that holds them in the cell membrane.

Desmogleins and desmocollins can pair as heterodimers. They form clusters through cis-interactions on the same cell and trans-interactions between adjacent cells. In humans, there are four desmoglein isoforms and three desmocollin isoforms, and their cytoplasmic domains can differ in length because of alternative splicing.

Inside the cell, the cadherins bind armadillo family proteins called plakoglobin and plakophilin. These proteins form a dense plaque. The plaque then recruits desmoplakin, the most abundant protein in a desmosomal complex. Desmoplakin uses its carboxy-terminal plakin repeats to bind intermediate filaments, which connects the desmosome to the cytoskeleton.

Problems with desmosomes often show up in the heart and skin, where cells face high mechanical strain. Mutations in desmoglein genes can cause epidermal thickening and arrhythmogenic right ventricular cardiomyopathy, or ARVC. Autoantibodies against desmosomal cadherins can also cause pemphigus vulgaris, an autoimmune disease that leads to epidermal blisters because cell-cell adhesion becomes weak.

Desmosomes and Cell Anchoring

cell adhesion molecules - types and functions

cell-adhesion-molecules-types-and-functions

Cell Adhesion Molecules in Tissues

Cell adhesion molecules help cells stick together and work as part of a tissue. They give multicellular organisms physical strength and also help cells send signals across the plasma membrane. These proteins are important for coordinated activity in tissues and organ systems. CAMs are grouped into four main families: integrins, cadherins, selectins, and immunoglobulin-like CAMs, or IgCAMs. Each family supports cell interactions in a different way. Some CAMs help cells attach firmly, while...

structure of cadherins

Cadherins in Cell Junctions and Disease

Cadherins are cell adhesion proteins that help cells stick together and build tissues. Their name comes from their calcium-dependent binding. Early examples were named E-cadherin, P-cadherin, and N-cadherin after the epithelial, placental, and neuronal cells where they were first found. Classical cadherins share similar sequences and structures. Other cadherins, including those involved in cell signaling, are grouped as non-classical cadherins. This diversity comes from multiple cadherin genes...

cadherins in tissue organization

Cadherins Guide Cell Sorting in Tissues

Cadherins help cells sort into the right tissues during development. They are a large superfamily of cell adhesion molecules, with more than 180 variants. Different tissues express specific cadherin combinations, which helps cells recognize and stick to the right neighbors. Cadherins usually bind to the same or a closely related cadherin on another cell. This is called homophilic binding. Because of this matching behavior, cells of the same type tend to group together and form clusters that...

catenins

Catenins in Cell Junctions and Signaling

Catenins help cells stay connected and also support signaling inside the body. These proteins act as linker proteins in cell junction complexes because they have multiple binding domains and flexible structures. Most catenins contain a protein sequence called the armadillo repeat, so they are also known as armadillo proteins. Alpha-catenin is the main exception. Catenins bind to cell adhesion molecules such as cadherins. They then connect cadherins to cytoskeletal proteins, and the exact link...

selectins

Selectins in Immune Cell Capture

Selectins help cells make short, temporary contacts during immune responses. They are cell adhesion molecules that bind carbohydrate ligands on other cells. These rod-like proteins have a long outside region and end in a lectin domain, which binds carbohydrates only when calcium ions are present. Three main selectins are known: L-selectin, P-selectin, and E-selectin. Their names reflect where they are found and how long they are. This tissue specificity helps explain why selectins act in...

immunoglobulin-like cell adhesion molecules

immunoglobulin-like-cell-adhesion-molecules

Ig-CAMs in Neural Growth and Disease

Ig-CAMs, or immunoglobulin-like cell adhesion molecules, are cell surface glycoproteins in the immunoglobulin superfamily. They contain immunoglobulin domains, and some also have fibronectin type III domains. Their Ig domains can be glycosylated to different degrees. These molecules can bind to the same kind of Ig-CAM or to other ligands, such as integrins. Most Ig-CAMs help cells stick together only for a short time. The nectin class is different because it helps form stable junctions,...

overview of cell-cell junctions

Cell Junctions in Tissues

Cell junctions help hold tissues together and let neighboring cells communicate. In multicellular organisms, the three-dimensional shape of tissues depends on interactions between cells and the extracellular matrix. Cell-cell junctions are specialized structures made of multi-protein complexes on one cell that connect with similar complexes on another cell. These junctions are grouped into three main types based on function: occluding, anchoring, and gap junctions. Tight junctions, also called...

adherens junctions

Cell Adhesion in Tissues and Infection

Adherens junctions are strong cell-to-cell anchors that help tissues stay connected. These anchoring junctions work with desmosomes, another type of junction that holds adjacent cells together. In many epithelial and endothelial tissues, adherens junctions form a continuous adhesion belt. In heart muscle, they often appear as clusters that help cells work together for coordinated contraction. Adherens junctions are also dynamic, especially in the cells that line blood vessels. These...

tension response at adherens junctions

How Adherens Junctions Sense Force

Adherens junctions help cells stay attached while they respond to mechanical force. These multi-protein complexes can adapt to tensile force and shear stress. When the force changes, mechanosensory proteins in the junction can change shape. This process is called mechanotransduction. Alpha-catenin is one of the main force-sensing proteins in adherens junctions. It is an allosteric protein, which means its shape affects how it works. Alpha-catenin has three VH, or vinculin homology, domains...

desmosomes

Desmosomes are cell junctions that anchor cells together. They are button-like structures that create an interlinked network of intermediate filaments across cells. This strong link helps tissues resist mechanical stress and maintain tissue integrity. Desmosomes are built from multiple proteins. Their core includes desmosomal cadherins, which are adhesion proteins that bind to cadherins on neighboring cells. The two desmosomal cadherins are desmogleins and desmocollins. These transmembrane...

tight junctions

Tight Junction Proteins and Cell Barriers

Tight junction proteins form seals between neighboring cells. These molecular barriers help stop fluids, ions, and other small solutes from leaking across body cavities and tissue compartments in multicellular organisms. They are an important part of cell barriers in places where controlled movement matters. The main tight junction proteins are claudin and occludin. Other proteins include tricellulin and JAM, which stands for junctional adhesion molecule. Most of these proteins are 4-pass...

gap junctions

Cell Communication Through Gap Junctions

Gap junctions let adjacent animal cells share small molecules, ions, and secondary messengers. These communication channels connect the cytoplasm of neighboring cells. They are built from many molecular channels, often from a few hundred to thousands in one junction. Each channel is made of two halves called connexon hemichannels. A connexon is a hexamer, meaning it is formed by six transmembrane connexin proteins. The six proteins arrange in a ring and create a pore in the center. One...

key terms and concepts

Learn how desmosomes hold cells together, link to intermediate filaments, and why defects can affect skin and heart tissue in humans.

Desmosomes and Cell Anchoring | JoVE K12

cell biology

JoVE Core Cell Biology illustrates complex cellular processes in easy-to-understand yet comprehensive animated video lessons. This collection focuses on the essential components and functions of cells, including organelles and membranes, cellular transport, and cell energetics. It is complemented by scientist-in-action videos that demonstrate the application of cell biology in real-world research experiments.

Desmosomes and Cell Anchoring

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