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Questions and Answers

What is the result of a severe desmoglein 3 deficiency?

  • Formation of desmosomes
  • Formation of hemidesmosomes
  • Blistering of the skin with leakage of body fluids (correct)
  • Increased cell-cell adhesion
  • Which type of intermediate filaments is found in epithelial cells?

  • Desmin filaments
  • Laminin filaments
  • Keratin filaments (correct)
  • Actin filaments
  • What is the function of anchoring junctions?

  • To regulate cell division
  • To regulate cell signaling pathways
  • To attach cells to each other or to the matrix (correct)
  • To provide mechanical support to cells
  • Which type of junctions is involved in cell-cell adhesion?

    <p>Desmosomes</p> Signup and view all the answers

    What is the function of hemidesmosomes?

    <p>To attach cells to the matrix</p> Signup and view all the answers

    Which protein is involved in the attachment of intermediate filaments to hemidesmosomes?

    <p>Plectin</p> Signup and view all the answers

    What is the consequence of autoantibodies attacking type XVII collagen in bullous pemphigoid?

    <p>Blistering of the skin</p> Signup and view all the answers

    What is the consequence of mutations in plectin?

    <p>Skin blisters associated with late-onset muscular dystrophy</p> Signup and view all the answers

    What is the function of desmoglein and desmocollin?

    <p>To bind to anchor proteins and intermediate filaments</p> Signup and view all the answers

    What is the result of desmosome disruption in skin autoimmune diseases like Pemphigus?

    <p>The skin epithelial cells are separated</p> Signup and view all the answers

    What type of proteins are desmoglein and desmocollin?

    <p>Cadherin proteins</p> Signup and view all the answers

    Where are desmosomes most abundant?

    <p>Tissues exposed to mechanical stress</p> Signup and view all the answers

    What is the role of desmoplakin, plakoglobin, and plakophilin?

    <p>To link cadherin proteins to intermediate filaments</p> Signup and view all the answers

    What is the purpose of desmosomes?

    <p>To hold cells together</p> Signup and view all the answers

    What is the result of desmoglein and desmocollin binding to anchor proteins?

    <p>The binding of cells to intermediate filaments</p> Signup and view all the answers

    Where are desmosomes found?

    <p>In many tissues, especially in skin and heart muscle</p> Signup and view all the answers

    What is the main function of anchoring junctions?

    <p>To anchor cells to the extracellular matrix or to other cells</p> Signup and view all the answers

    What is the role of integrins in focal adhesions?

    <p>To bind to laminin protein in the basal lamina</p> Signup and view all the answers

    What is the consequence of mutations in plectin?

    <p>Skin blisters</p> Signup and view all the answers

    What is the function of hemidesmosomes?

    <p>To facilitate the adhesion of cells to the basal lamina</p> Signup and view all the answers

    What is the role of the cytoplasmic domain of integrins in focal adhesions?

    <p>To bind to actin filaments</p> Signup and view all the answers

    What type of collagen is attacked by autoantibodies in bullous pemphigoid?

    <p>Type XVII collagen</p> Signup and view all the answers

    What is the main function of desmoglein and desmocollin?

    <p>To facilitate cell-cell adhesion</p> Signup and view all the answers

    What is the characteristic of hemidesmosomes?

    <p>They have a single dense plaque on the cytoplasmic surface</p> Signup and view all the answers

    What is the primary function of microvilli?

    <p>To increase the surface area of the cell for absorption</p> Signup and view all the answers

    What are the proteins that cross-link actin filaments in microvilli?

    <p>Fimbrin and villin</p> Signup and view all the answers

    What is the characteristic of glycocalix around microvilli?

    <p>It is thicker</p> Signup and view all the answers

    What is the function of cilia in epithelial cells?

    <p>To move the fluid over the surface of the cell</p> Signup and view all the answers

    Where are cilia typically found in the human body?

    <p>In the upper respiratory tract and uterine tubes</p> Signup and view all the answers

    What is the structure of cilia composed of?

    <p>Microtubules</p> Signup and view all the answers

    What is the composition of a triplet microtubule in a basal body?

    <p>One complete microtubule and two incomplete microtubules</p> Signup and view all the answers

    What is the structure that extends from the basal body into the apical cytoplasm?

    <p>Rootlet</p> Signup and view all the answers

    What is the function of basal bodies in the organization of cilia and flagella?

    <p>They organize the axoneme microtubules</p> Signup and view all the answers

    What is the difference between a basal body and a centriole?

    <p>Basal bodies have accessory structures such as basal foot and rootlet</p> Signup and view all the answers

    What is the significance of ciliary movement during development?

    <p>It is involved in the organization of internal organs</p> Signup and view all the answers

    What is the characteristic of cilia in patients with Kartagener's Syndrome?

    <p>They are immobile</p> Signup and view all the answers

    What is the role of nodal cilia in development?

    <p>They help in the organization of internal organs</p> Signup and view all the answers

    What is the main function of the flagellum in mammalian sperm?

    <p>To propel the sperm during fertilization</p> Signup and view all the answers

    What is the characteristic of microtubules?

    <p>They are hollow tube-like structures composed of 13 protofilaments</p> Signup and view all the answers

    What is the significance of the helical beating of cilia at the node?

    <p>It is responsible for the origin of left-right asymmetry</p> Signup and view all the answers

    What is the main difference between flagella and cilia?

    <p>The type of movement they exhibit</p> Signup and view all the answers

    What is the function of sterocilia?

    <p>They have no motility</p> Signup and view all the answers

    What is the composition of protofilaments in microtubules?

    <p>Tubulin alpha and beta subunits</p> Signup and view all the answers

    What is the function of the basal body in a ciliated cell?

    <p>To organize the formation of cilia and flagella</p> Signup and view all the answers

    What is the structure of the axoneme in flagella and cilia?

    <p>9+2 arrangement</p> Signup and view all the answers

    What is the characteristic of centrioles?

    <p>They are involved in the formation of cilia and flagella</p> Signup and view all the answers

    What type of anchoring junctions are responsible for binding cells to the extracellular matrix?

    <p>Focal adhesions</p> Signup and view all the answers

    What is the function of the cytoplasmic domain of integrins in focal adhesions?

    <p>Bind indirectly to actin filaments</p> Signup and view all the answers

    Which protein is involved in the attachment of intermediate filaments to hemidesmosomes?

    <p>Plectin</p> Signup and view all the answers

    What is the consequence of mutations in plectin?

    <p>Skin blisters</p> Signup and view all the answers

    Which type of collagen is attacked by autoantibodies in bullous pemphigoid?

    <p>Type XVII collagen</p> Signup and view all the answers

    What is the characteristic of hemidesmosomes?

    <p>Single dense plaque</p> Signup and view all the answers

    What is the role of integrins in focal adhesions?

    <p>Bind to the extracellular matrix</p> Signup and view all the answers

    What type of anchoring junctions are involved in cell-cell adhesion?

    <p>Desmosomes</p> Signup and view all the answers

    What is the main role of cytoplasmic plaque proteins in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    Which of the following is NOT a cytoplasmic plaque protein?

    <p>Desmocollin</p> Signup and view all the answers

    What is the main function of desmoplakin in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    Which of the following cytoplasmic plaque proteins is involved in the attachment of intermediate filaments to desmosomes?

    <p>All of the above</p> Signup and view all the answers

    What is the consequence of disrupting the cytoplasmic plaque proteins in desmosomes?

    <p>Weakened cell adhesion</p> Signup and view all the answers

    Which of the following proteins is involved in the attachment of cadherins to intermediate filaments?

    <p>Desmoplakin</p> Signup and view all the answers

    What is the role of plakophilin in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    What is the function of fibrin in blood clots?

    <p>To form an elastic network</p> Signup and view all the answers

    Which of the following is a characteristic of desmosomes?

    <p>They contain plaque-shaped structures on the cytoplasmic face</p> Signup and view all the answers

    What is the enzyme that cleaves fibrinogen to form fibrin?

    <p>Thrombin</p> Signup and view all the answers

    What is the function of von Willebrand factor in thrombus formation?

    <p>To mediate platelet adhesion</p> Signup and view all the answers

    What is the site of storage of von Willebrand factor in endothelial cells?

    <p>Weibel–Palade bodies</p> Signup and view all the answers

    What is the enzyme that cleaves fibrin to disperse blood clots?

    <p>Plasmin</p> Signup and view all the answers

    What is the precursor molecule of plasmin?

    <p>Plasminogen</p> Signup and view all the answers

    What is the function of fibrin in promoting angiogenesis?

    <p>By binding to vascular endothelial growth factor</p> Signup and view all the answers

    What is the role of tissue plasminogen activator in fibrinolysis?

    <p>To activate plasminogen</p> Signup and view all the answers

    What is the purpose of the plaque proteins in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    Which type of junctions are desmosomes?

    <p>Desmosomes are anchoring junctions</p> Signup and view all the answers

    What is the role of desmoglein and desmocollin in desmosomes?

    <p>To link adjacent cells together</p> Signup and view all the answers

    In which tissues are desmosomes most abundant?

    <p>Skin and heart muscle</p> Signup and view all the answers

    What is the result of desmosome disruption in skin autoimmune diseases like Pemphigus?

    <p>Disruption of skin epithelial cells</p> Signup and view all the answers

    What is the role of desmoplakin in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    What type of filaments are attached to desmosomes?

    <p>Intermediate filaments</p> Signup and view all the answers

    What is the function of desmosomes in tissues?

    <p>To provide mechanical strength</p> Signup and view all the answers

    What type of proteins do Integrins bind to in the basal lamina?

    <p>Laminin</p> Signup and view all the answers

    What is the characteristic feature of Hemidesmosomes?

    <p>Single dense plaque on the cytoplasmic surface</p> Signup and view all the answers

    Which type of intermediate filaments is attached to Hemidesmosomes?

    <p>Keratin filaments</p> Signup and view all the answers

    What is the consequence of autoantibodies attacking Type XVII Collagen in Bullous Pemphigoid?

    <p>Skin blisters</p> Signup and view all the answers

    Which protein is involved in the attachment of intermediate filaments to Hemidesmosomes?

    <p>Plectin</p> Signup and view all the answers

    What is the function of Hemidesmosomes?

    <p>Cell-matrix adhesion</p> Signup and view all the answers

    What is the structure that connects an epithelial cell to the basal lamina?

    <p>Hemidesmosome</p> Signup and view all the answers

    What is the consequence of mutations in Plectin?

    <p>Muscular dystrophy with skin blisters</p> Signup and view all the answers

    What is the function of desmosomes in cells?

    <p>To provide mechanical binding between adjacent cells</p> Signup and view all the answers

    What type of filaments are anchored to desmosomes?

    <p>Intermediate filaments</p> Signup and view all the answers

    What is the structure of a desmosome?

    <p>A button-like point of tight adhesion</p> Signup and view all the answers

    What is the role of desmosomes in vertebrate development?

    <p>To form the neural tube</p> Signup and view all the answers

    What is the function of adherens junctions?

    <p>To provide strong adhesion between adjacent cells</p> Signup and view all the answers

    What is the role of α-catenin in adherens junctions?

    <p>To link actin filaments to cadherins</p> Signup and view all the answers

    What is the structure of an adherens junction?

    <p>A complex of actin filaments, cadherins, and α-catenin</p> Signup and view all the answers

    What is the function of vinculin in adherens junctions?

    <p>To anchor actin filaments to the cell membrane</p> Signup and view all the answers

    What is the effect of autoantibodies attacking type XVII collagen in bullous pemphigoid?

    <p>Causing skin blisters</p> Signup and view all the answers

    What is the result of a severe desmoglein 3 deficiency in the skin?

    <p>Blistering of the skin with leakage of body fluids</p> Signup and view all the answers

    What is the consequence of mutations in plectin?

    <p>Skin blisters</p> Signup and view all the answers

    Which type of junction is responsible for attaching epithelial cells to the basal lamina?

    <p>Hemidesmosomes</p> Signup and view all the answers

    Which type of anchoring junctions are responsible for binding cells to the extracellular matrix in skin?

    <p>Focal adhesions</p> Signup and view all the answers

    What is the function of integrins in hemidesmosomes?

    <p>To bind to laminin protein in the basal lamina</p> Signup and view all the answers

    What is the role of integrins in focal adhesions in skin?

    <p>Binding to the extracellular matrix</p> Signup and view all the answers

    What is the function of hemidesmosomes in skin?

    <p>Cell-matrix adhesion</p> Signup and view all the answers

    What is the consequence of autoantibodies attacking type XVII collagen in bullous pemphigoid?

    <p>Skin blistering</p> Signup and view all the answers

    Which protein is involved in the attachment of intermediate filaments to hemidesmosomes in skin?

    <p>Plectin</p> Signup and view all the answers

    What is the result of mutations in plectin?

    <p>Late-onset muscular dystrophy</p> Signup and view all the answers

    What is the characteristic of hemidesmosomes?

    <p>Single dense plaque on the cytoplasmic surface</p> Signup and view all the answers

    What is the characteristic of hemidesmosomes in skin?

    <p>Having a single dense plaque</p> Signup and view all the answers

    Which skin disease is caused by autoantibodies attacking type XVII collagen in the basal lamina?

    <p>Bullous pemphigoid</p> Signup and view all the answers

    What type of intermediate filaments are involved in hemidesmosomes?

    <p>Keratin filaments</p> Signup and view all the answers

    What is the function of plectin in hemidesmosomes?

    <p>To anchor intermediate filaments to hemidesmosomes</p> Signup and view all the answers

    What is the result of desmosome disruption in skin autoimmune diseases like Pemphigus?

    <p>The skin epithelial cells become less adherent to each other</p> Signup and view all the answers

    Which protein is responsible for the mechanical binding of cells in desmosomes?

    <p>Desmoglein</p> Signup and view all the answers

    What is the characteristic of desmosomes in skin autoimmune diseases like Pemphigus?

    <p>Weaker adhesion between cells</p> Signup and view all the answers

    What is the consequence of autoantibodies binding to desmosomal cadherins in Pemphigus?

    <p>The skin epithelial cells become less adherent to each other</p> Signup and view all the answers

    What is the role of plaque proteins in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    What is the characteristic of desmosomes in tissues that are exposed to mechanical stress?

    <p>They are more abundant</p> Signup and view all the answers

    What is the function of desmoplakin in desmosomes?

    <p>To provide attachment sites for intermediate filaments</p> Signup and view all the answers

    What is the consequence of desmosome disruption in skin autoimmune diseases?

    <p>Skin epithelial cells become less adherent to each other</p> Signup and view all the answers

    Study Notes

    Anchoring Junctions

    • Anchoring junctions can be subclassified according to the cytoskeletal element that is involved.
    • There are two types of anchoring junctions:
      • Actin filament attachment sites
      • Intermediate filament attachment sites

    Actin Filament Attachment Sites

    • Cell-cell junction (ADHERENS JUNCTIONS)
    • Cell-matrix junctions (FOCAL ADHESIONS)

    Intermediate Filament Attachment Sites

    • Cell-cell junctions (DESMOSOMES)
    • Cell-matrix junctions (HEMIDESMOSOMES)

    Desmosomes

    • Strongest points of cell adhesion that provide mechanical binding
    • Most abundant in tissues that are exposed to mechanical stress (epidermis of the skin, heart muscle)
    • Cell-to-cell binding depends on cadherin family of proteins called desmoglein and desmocollin
    • Desmosomes contain plaque-shaped structures on the cytoplasmic face of the junction, which provide attachment sites for intermediate filaments
    • Plaque proteins are plakoglobins, desmoplakins, and plakophilins
    • Two types of cadherins (desmoglein and desmocollin) link adjacent cells together, and cytoplasmic plaque (desmoplakin, plakoglobin, and plakophilin) links the cadherins
    • Desmosomes give tissues mechanical strength
    • Desmosomes are found in many tissues, especially abundant in skin (epidermis), heart muscle, and the neck of the uterus

    Importance of Desmosome Junctions

    • Demonstrated by some of the skin autoimmune diseases, such as PEMPHIGUS
    • Affected individuals make antibodies against their own desmosomal cadherins, which bind to and disrupt the desmosomes that hold their skin epithelial cells together

    Hemidesmosomes

    • Resemble desmosomes morphologically
    • Cell uses hemidesmosomes to attach to the basal lamina
    • Cell-to-matrix adhesion proteins, such as INTEGRINS, are involved in hemidesmosomes
    • Hemidesmosomes have only a single dense plaque on the cytoplasmic surface of the hemidesmosome (hemi=half) that anchors loops of intermediate filaments
    • Schematic model of hemidesmosome connecting an epithelial cell to the basal lamina
    • Extracellular domains of the integrins bind to laminin protein in the basal lamina, and an intracellular domain binds to an anchor protein (plectin) that binds to keratin intermediate filaments
    • In a blistering skin disease called bullous pemphigoid, autoantibodies attack type XVII collagen
    • Mutations in plectin cause skin blisters associated with late-onset muscular dystrophy

    Focal Adhesions

    • Bind the cells to the extracellular matrix
    • Cell-to-matrix adhesion proteins, such as INTEGRINS, are responsible for the binding to the matrix
    • Cytoplasmic domain of the integrin binds indirectly to actin filaments
    • Integrin's extracellular domains bind to components of the extracellular matrix, while the cytoplasmic tail of the β subunit binds indirectly to actin

    Microvillus Structure

    • A bundle of parallel actin filaments extend 0.5μ down into the apical cytoplasm and enter into the terminal web
    • Actin filaments are cross-linked into closely packed bundles by actin-bundling proteins, villin, and fimbrin, forming the core of a microvillus
    • Actin filaments are attached to the plasma membrane by lateral arms consisting of myosin I and calmodulin
    • Glycocalix is thicker around the microvilli, forming a striated border, and is PAS+

    Cilia and Flagella

    • Cilia are eyelash or hair-like processes from the cell surface, motile, and longer than microvilli (5-10μ long, 0.2μ in diameter)
    • Under the electron microscope, they have a complex internal structure composed of microtubules
    • 250 or more cilia are found in each cell, arranged in parallel rows, and function to move fluid over the surface of the cell
    • Found in epithelial cells of the upper respiratory tract, uterine tubes (oviducts), and efferent ducts (Ductus efferentes)

    Basal Body Structure

    • At the base of a cilium, a central pair of single microtubules terminates
    • Each of the peripheral doublets is continuous with a triplet microtubule of the basal body
    • Three microtubules are fused together and form a triplet microtubule
    • Basal body resembles a centriole but contains some accessory structures such as basal foot and rootlet

    Cilia Function

    • Ciliary movement is important during development
    • Dynein arms are absent from cilia in patients with Kartagener's Syndrome
    • Nodal Cilia cannot move during development, and internal organs cannot be located at their normal positions
    • Helical beating of cilia at the node, and the origins of left-right asymmetry

    Flagella Structure and Function

    • Flagellum propels sperm, is longer than cilia (100-200 μ), and has the same internal structure as cilia (Axoneme 9+2)
    • Has a different type of movement (undulating wave type of movement) and is less in number (one or two in a single cell)
    • Mammalian spermium contains 9 additional dense fibers around the axoneme (9+9+2), which serves a protective function

    Microtubule Structure

    • Microtubules are hollow tube-like or pipe-like structures
    • The wall of the microtubule is composed of 13 protofilaments
    • Protofilaments are composed of Tubulin subunits (dimer), which consist of Tubulin α and Tubulin β

    Gap Junctions

    • Site of firm adhesion of cells
    • Allow free interchange of substances
    • Important role in regulation of intrauterine development and differentiation
    • Coordinate function among groups of cells

    Anchoring Junctions

    • Two types: Hemidesmosomes and Focal Adhesions

    Hemidesmosomes

    • Have a single dense plaque on the cytoplasmic surface
    • Anchor loops of intermediate filaments
    • Integrins bind to laminin protein in the basal lamina
    • Intracellular domain binds to an anchor protein (plectin) that binds to keratin intermediate filaments
    • Found in epithelial cells, attach to basal lamina
    • Mutations in plectin cause skin blisters
    • Autoantibodies attack type XVII collagen in bullous pemphigoid, a blistering skin disease

    Focal Adhesions

    • Bind cells to the extracellular matrix
    • Cell-matrix adhesion proteins; integrins responsible for binding to the matrix
    • Cytoplasmic domain of the integrin binds indirectly to actin filaments
    • Integrin's extracellular domains bind to components of extracellular matrix
    • Cytoplasmic tail of the β subunit binds indirectly to actin

    Adhesion Proteins

    • Fibrin: a major ECM component of blood clots
    • Forms an elastic network to which cells and other ECM components bind
    • Polymerization of fibrin to form the network occurs when its precursor molecule fibrinogen is cleaved by the enzyme thrombin
    • Fibrin has binding interactions with various extracellular components, including fibronectin, heparin, growth factors, and cytokines
    • Fibrinolysis is mediated by plasmin, which cleaves fibrin

    Von Willebrand Factor

    • Plays a key role in the major response of platelets to vascular injury by mediating the initiation and progression of thrombus formation
    • Enables cell adhesion to develop by forming a bridge between collagen in the vessel wall and blood platelets

    Desmosomes

    • Strongest points of cell adhesion that provide mechanical binding
    • Most abundant in tissues that are exposed to mechanical stress (epidermis of the skin, heart muscle)
    • Cell-to-cell binding depends on cadherin family of proteins called desmoglein and desmocollin
    • Contain plaque-shaped structures on the cytoplasmic face of the junction which provide attachment sites for intermediate filaments
    • Plaque proteins are plakoglobins, desmoplakins, and plakophilins
    • Found in many tissues, especially abundant in skin, heart muscle, and the neck of the uterus
    • Importance of desmosome junctions is demonstrated by some skin autoimmune diseases, such as pemphigus, where affected individuals make antibodies against their own desmosomal cadherins

    Desmosomes

    • Strongest points of cell adhesion that provide mechanical binding
    • Most abundant in tissues exposed to mechanical stress (epidermis of the skin, heart muscle)
    • Contain plaque-shaped structures on the cytoplasmic face of the junction, providing attachment sites for intermediate filaments
    • Plaque proteins include plakoglobins, desmoplakins, and plakophilins
    • Two types of cadherins (desmoglein and desmocollin) link adjacent cells together
    • Cytoplasmic plaque (desmoplakin, plakoglobin, and plakophilin) links the cadherins to intermediate filaments

    Importance of Desmosomes

    • Desmosomal cadherins are targeted in autoimmune diseases such as Pemphigus, leading to blistering of the skin
    • Affected individuals make antibodies against their own desmosomal cadherins, disrupting desmosomes and causing skin epithelial cells to separate

    Intermediate Filaments

    • Types of intermediate filaments include keratin filaments in epithelial cells and desmin filaments in heart and muscle cells
    • Intermediate filaments can be subclassified according to the cytoskeletal element involved

    Anchoring Junctions

    • Subclassified into two types:
      • Intermediate filament attachment sites
        • Cell-cell junctions (desmosomes)
        • Cell-matrix junctions (hemidesmosomes)
      • Actin filament attachment sites
        • Cell-cell junctions (adherens junctions)
        • Cell-matrix junctions (focal adhesions)

    Hemidesmosomes

    • Resemble desmosomes morphologically
    • Cell uses hemidesmosomes to attach to the basal lamina
    • Contain a single dense plaque on the cytoplasmic surface that anchors loops of intermediate filaments
    • Schematic model shows an epithelial cell attached to the basal lamina via hemidesmosomes
    • Autoantibodies attack type XVII collagen in bullous pemphigoid, a blistering skin disease
    • Mutations in plectin cause skin blisters associated with late-onset muscular dystrophy

    Adherens Junctions

    • Actin filament bundles are attached by intracellular anchor proteins to cadherins
    • Cadherins are transmembrane proteins that bind to those of adjacent cells, tying actin filament bundles together
    • Adherens junctions in the form of adhesion belts between epithelial cells in the small intestine
    • Role in early development: controlled contraction of actin filament bundles causes epithelial cells to narrow and form a tube in vertebrate development

    Anchoring Junctions

    • Anchoring junctions can be subclassified according to the cytoskeletal element involved
    • Two types of anchoring junctions:
      • Actin filament attachment sites
        • Cell-cell junctions (ADHERENS JUNCTIONS)
        • Cell-matrix junctions (FOCAL ADHESIONS)
      • Intermediate filament attachment sites
        • Cell-cell junctions (DESMOSOMES)
        • Cell-matrix junctions (HEMIDESMOSOMES)

    Desmosomes

    • Desmosomes are strongest points of cell adhesion that provide mechanical binding
    • Most abundant in tissues that are exposed to mechanical stress (Epidermis of the skin, heart muscle)
    • Cell to cell binding depends on cadherin family of proteins called desmoglein and desmocollin
    • Desmosomes contain plaque-shaped structures on the cytoplasmic face of the junction which provide attachment sites for intermediate filaments
    • Plaque proteins: plakoglobins, desmoplakins, plakophilins

    Hemidesmosomes

    • Hemidesmosomes resemble desmosomes morphologically
    • Hemidesmosomes have only a single dense plaque on the cytoplasmic surface of the hemidesmosome (hemi=half) that anchors loops of intermediate filaments
    • Hemidesmosomes connect epithelial cells to the basal lamina
    • Integrin (α6β4) and type XVII collagen (also called BPAG2) attach to the basal lamina

    Importance of Desmosomes and Hemidesmosomes

    • In a blistering skin disease called bullous pemphigoid, autoantibodies attack type XVII collagen
    • Mutations in plectin cause skin blisters associated with late-onset muscular dystrophy
    • Cataract, heart malformations, and skin blistering are examples of diseases related to desmosome and hemidesmosome dysfunction

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    This quiz covers the basics of intermediate filaments and anchoring junctions in cells, including types of filaments and their locations in different cell types.

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