118 Questions
What is the function of desmoglein and desmocollin in desmosomes?
To link adjacent cells together
Which proteins bind to desmoglein and desmocollin to link them to intermediate filaments?
Plakoglobins and desmoplakins
What is the effect of autoimmune disease pemphigus on desmosomes?
It disrupts the desmosomes that hold skin epithelial cells together
What is the primary function of desmosomes?
To provide mechanical strength to tissues
Which of the following tissues is desmosomes most abundant in?
Epithelial tissue
What is the role of plaque proteins in desmosomes?
They provide attachment sites for intermediate filaments
What is the function of desmoplakin in desmosomes?
It provides attachment sites for intermediate filaments
What type of proteins are desmoglein and desmocollin?
Cadherin proteins
What is the role of Cx43, Cx46, and Cx50/α8 in the development of certain diseases?
All of the above
What type of junction allows for the free interchange of substances between cells?
Gap junctions
What is the result of a defect in Desmoglein 3?
Severe blistering of the skin
What is the role of hemidesmosomes in the cell?
They are involved in cell-matrix adhesion
What type of intermediate filaments are found in epithelial cells?
Keratin filaments
What protein binds to laminin in the basal lamina and to keratin intermediate filaments in hemidesmosomes?
Integrin
What type of anchoring junction involves actin filament attachment sites?
Adherens junctions
What is the result of autoantibodies attacking type XVII collagen in the basal lamina?
Blistering skin disease
What type of anchoring junction is involved in the attachment of actin filaments to the extracellular matrix?
Focal adhesions
What is the function of hemidesmosomes in cells?
Cell-matrix adhesion
What is the difference between hemidesmosomes and desmosomes?
Hemidesmosomes have a single dense plaque
What is the name of the protein that is mutated in skin blistering diseases?
Plectin
What type of anchoring junction is involved in the attachment of intermediate filaments to the basal lamina?
Hemidesmosomes
What protein is associated with the basal lamina in hemidesmosomes?
Laminin
What is the consequence of autoantibodies attacking type XVII collagen?
Bullous pemphigoid
What is the result of mutations in plectin?
Late-onset muscular dystrophy with skin blisters
What is the main component of a microvillus?
Actin filaments
What is the function of villin and fimbrin in microvilli?
To cross-link actin filaments into bundles
Where are cilia typically found?
On the surface of epithelial cells of the uterine tubes and respiratory tract
What is the function of cilia?
To move the fluid over the surface of the cell
What is the structure of cilia composed of?
Microtubules
What is the thickness of the glycocalix around microvilli?
Thicker
What is the composition of a triplet microtubule in a basal body?
One complete microtubule fused to two incomplete microtubules
What is the function of basal bodies in cilia and flagella?
To organize the axoneme microtubules
What is the difference between a basal body and a centriole?
Basal bodies have accessory structures such as basal foot and rootlet
What is the result of a defect in the formation of basal bodies in cilia during development?
Internal organs cannot be located at their normal positions
What is the structure that extends from the basal body into the apical cytoplasm?
Rootlet
How many sets of triplet microtubules are found in a basal body?
Nine
What is the movement pattern of cilia at the node during development?
Helical
What is the term for the fibrous material that extends laterally from the basal body?
Basal foot
What is the structure of microtubules?
Hollow tube-like structures composed of 13 protofilaments
What is the function of the basal body in a ciliated cell?
To organize the microtubules of cilia
What is the characteristic feature of stereocilia?
They are long and irregular microvilli
What is the difference between cilia and flagella?
Cilia have a different type of movement than flagella
What is the function of centrioles?
To organize the microtubules of cilia and flagella
What is the structure of flagellum of mammalian spermium?
9+2 microtubule arrangement with 9 additional dense fibers
What is the difference between the movement of flagella and cilia?
Flagella move with an undulating wave-like motion, while cilia move with a beating motion
What is the function of the node in embryonic development?
To produce signal proteins that establish left-right asymmetry
What is the primary function of anchoring junctions?
To provide mechanical support and adhesion between cells or between cells and the extracellular matrix
What is the type of anchoring junction that involves actin filament attachment sites?
Focal adhesions
What is the result of a defect in the protein plectin?
Skin blisters
What is the type of protein that binds to laminin in the basal lamina and to keratin intermediate filaments in hemidesmosomes?
Integrin
What is the type of anchoring junction that involves intermediate filament attachment sites?
Hemidesmosomes
What is the function of the protein integrin in focal adhesions?
To bind to laminin in the basal lamina
What is the consequence of autoantibodies attacking type XVII collagen in the basal lamina?
Blistering skin disease
What is the type of cytoskeletal element involved in focal adhesions?
Actin filaments
What is the primary function of fibrin in blood clots?
To form an elastic network to which cells and other ECM components bind
What is the enzyme responsible for cleaving fibrinogen to form fibrin?
Thrombin
What is the function of von Willebrand Factor (vWF) in thrombus formation?
To form a bridge between collagen in the vessel wall and blood platelets
What is the precursor molecule of fibrin?
Fibrinogen
Which of the following proteins are found in the cytoplasmic plaque of desmosomes?
Plakoglobins, desmoplakins, and plakophilins
What is the enzyme responsible for cleaving plasminogen to form plasmin?
Tissue plasminogen activator
What is the role of plaque proteins in desmosomes?
To link cadherins to intermediate filaments
Which of the following proteins is NOT a component of the cytoplasmic plaque of desmosomes?
Laminin
What is the function of plasmin in the dispersal of blood clots?
To cleave fibrin and promote the dispersal of blood clots
What is the function of the cytoplasmic plaque in desmosomes?
To provide attachment sites for intermediate filaments
What is the binding site for fibrin?
All of the above
What is the structure of fibrinogen molecules?
Elongated structures 45 nm in length with two sets of αα, Bß, and γ chains linked by disulfide bonds
Which of the following is a characteristic of desmosomes?
They provide mechanical strength to the tissue
What is the consequence of autoantibodies attacking desmosomal cadherins in pemphigus?
The disruption of desmosomes and the loss of mechanical strength
Which of the following tissues is desmosomes most abundant in?
Epidermis and heart muscle
What is the function of desmosomes in tissues?
To provide mechanical strength to the tissue
What is the main function of desmosomes in cells?
To provide mechanical strength to tissues
What type of proteins are involved in the attachment of cadherins to intermediate filaments?
Desmoplakin and plakoglobin
What is the shape of the structures on the cytoplasmic face of the junction?
Plaque-shaped
Which of the following tissues is desmosomes most abundant in?
Skin (epidermis) and heart muscle
What is the result of autoantibodies attacking desmosomal cadherins?
Disruption of desmosomes leading to skin blistering
What is the function of desmoglein and desmocollin?
To link adjacent cells together
What is the location of desmoplakin, plakoglobin, and plakophilin in desmosomes?
In the plaque-shaped structures on the cytoplasmic face of the junction
What is the importance of desmosome junctions?
They provide mechanical strength to tissues
What is the primary function of adherens junctions?
To provide strong adhesion between adjacent cells and form attachment sites for actin filaments
What is the role of α-catenin and ß-catenin in adherens junctions?
To link the cytoplasmic domain of E-Cadherin to actin filaments
What is the result of the controlled contraction of actin filament bundles in adherens junctions during early development?
The formation of a neural tube
What is the function of desmosomes?
To provide mechanical binding between cells and act as the strongest points of cell adhesion
What type of anchoring junction involves intermediate filament attachment sites?
Desmosomes
What is the structure of desmosomes?
A button-like point of tight adhesion
What is the role of vinculin in adherens junctions?
To anchor actin filaments to cadherins
What is the function of adherens junctions in vertebrate development?
To play an important role in the formation of the neural tube
Which type of anchoring junctions is involved in the attachment of intermediate filaments to the basal lamina?
Hemidesmosomes
What is the result of autoantibodies attacking type XVII collagen in the basal lamina?
Bullous pemphigoid
Which protein binds to laminin in the basal lamina and to keratin intermediate filaments in hemidesmosomes?
Integrin
What is the result of mutations in plectin?
Both skin blisters and muscular dystrophy
What type of intermediate filaments are found in epithelial cells?
Keratin
What is the function of hemidesmosomes in cells?
Cell-matrix adhesion
What is the main difference between hemidesmosomes and desmosomes?
Hemidesmosomes attach to the basal lamina, while desmosomes attach to other cells
What is the result of a defect in Desmoglein 3?
Pemphigus vulgaris
Which type of skin disease is caused by autoantibodies attacking desmoglein 3?
Pemphigus vulgaris
What is the result of mutations in the plectin protein?
Skin blisters associated with late-onset muscular dystrophy
Which type of anchoring junction involves the attachment of intermediate filaments to the basal lamina?
Hemidesmosomes
What is the role of integrins in hemidesmosomes?
Binding to laminin protein in the basal lamina
What is the consequence of autoantibodies attacking type XVII collagen in the basal lamina?
Bullous pemphigoid
What type of intermediate filaments are found in epithelial cells?
Keratin filaments
What is the role of hemidesmosomes in the cell?
Attachment of cells to the basal lamina
What is the result of a defect in desmoglein 3?
Pemphigus vulgaris
What is the result of autoantibodies attacking type XVII collagen in the basal lamina?
Blisters in the skin
What is the consequence of mutations in plectin?
Skin blisters
Which of the following is associated with skin blistering diseases?
Plectin
What is the role of hemidesmosomes in bullous pemphigoid?
Cell-matrix adhesion
What is the result of a defect in the protein plectin?
Skin blisters
What is the role of integrin in hemidesmosomes?
Binding to laminin in the basal lamina
What is the type of anchoring junction involved in the attachment of intermediate filaments to the basal lamina?
Hemidesmosomes
What is the protein that binds to laminin in the basal lamina and to keratin intermediate filaments in hemidesmosomes?
Integrin
What is the result of autoimmune disease pemphigus on skin epithelial cells?
The desmosomes are disrupted, leading to skin epithelial cell separation.
Which protein is targeted by autoantibodies in pemphigus?
Desmoglein
What is the primary function of desmosomes in the skin?
To provide mechanical strength and adhesion between cells.
Which of the following tissues is desmosomes most abundant in?
All of the above
What is the role of plaque proteins in desmosomes?
To provide attachment sites for intermediate filaments.
Which type of cadherin protein is involved in desmosomes?
Desmoglein and desmocollin
What is the result of disruption of desmosomes in the skin?
Separation of skin epithelial cells
What is the importance of desmosome junctions demonstrated by?
Autoimmune disease pemphigus
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 and intermediate filament attachment sites.
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
- 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, plakophilins
- Desmosomes are found in many tissues, especially abundant in skin (epidermis), heart muscle, and the neck of the uterus
- Importance of desmosome junctions is demonstrated by some of the skin autoimmune diseases, such as pemphigus
Hemidesmosomes
- Resemble desmosomes morphologically
- Cell uses hemidesmosomes to attach to the basal lamina
- Have only a single dense plaque on the cytoplasmic surface of the hemidesmosome (hemi=half) that anchors loops of intermediate filaments
- Integrin (α6β4) and type XVII collagen (also called BPAG2) attach to the basal lamina
- 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; integrins 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 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)
- Intermediate filament attachment sites
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)
- Actin filament attachment sites
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
This quiz covers the structure and function of cells, including desmoglein, pemphigus vulgaris, and intermediate filaments, as well as anchoring junctions and their classification.
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