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Anchoring Fibrils
Anchoring fibrils (composed largely of type VII collagen) extend from the basal lamina of epithelial cells and attach to the lamina reticularis (also known as the reticular lamina) by wrapping around the reticular fiber ( collagen III) bundles. The basal lamina and lamina reticularis together make up the basement membrane. Anchoring fibrils are essential to the functional integrity of the dermoepidermal junction. Epidermolysis bullosa dystrophica Epidermolysis bullosa dystrophica, also known as Dystrophic EB (DEB) is a chronic skin condition caused when anchoring fibrils are abnormal, diminished, or absent. This causes a weak dermoepidermal junction, where the epidermis easily separates from the dermis causing much pain. This condition is caused by a mutation of COL7A1, the gene that codes for a type of collagen 7. See also *Epidermis (skin) The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and hypodermis. Th ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Collagen
Collagen () is the main structural protein in the extracellular matrix found in the body's various connective tissues. As the main component of connective tissue, it is the most abundant protein in mammals, making up from 25% to 35% of the whole-body protein content. Collagen consists of amino acids bound together to form a triple helix of elongated fibril known as a collagen helix. It is mostly found in connective tissue such as cartilage, bones, tendons, ligaments, and skin. Depending upon the degree of mineralization, collagen tissues may be rigid (bone) or compliant (tendon) or have a gradient from rigid to compliant (cartilage). Collagen is also abundant in corneas, blood vessels, the gut, intervertebral discs, and the dentin in teeth. In muscle tissue, it serves as a major component of the endomysium. Collagen constitutes one to two percent of muscle tissue and accounts for 6% of the weight of the skeletal muscle tissue. The fibroblast is the most common cell that crea ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Basal Lamina
The basal lamina is a layer of extracellular matrix secreted by the epithelial cells, on which the epithelium sits. It is often incorrectly referred to as the basement membrane, though it does constitute a portion of the basement membrane. The basal lamina is visible only with the electron microscope, where it appears as an electron-dense layer that is 20–100 nm thick (with some exceptions that are thicker, such as basal lamina in lung alveoli and renal glomeruli). Structure The layers of the basal lamina ("BL") and those of the basement membrane ("BM") are described below: Anchoring fibrils composed of type VII collagen extend from the basal lamina into the underlying reticular lamina and loop around collagen bundles. Although found beneath all basal laminae, they are especially numerous in stratified squamous cells of the skin. These layers should not be confused with the lamina propria, which is found outside the basal lamina. Basement membrane The basement membra ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Epithelial Cells
Epithelium or epithelial tissue is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. It is a thin, continuous, protective layer of compactly packed cells with a little intercellular matrix. Epithelial tissues line the outer surfaces of organs and blood vessels throughout the body, as well as the inner surfaces of cavities in many internal organs. An example is the epidermis, the outermost layer of the skin. There are three principal shapes of epithelial cell: squamous (scaly), columnar, and cuboidal. These can be arranged in a singular layer of cells as simple epithelium, either squamous, columnar, or cuboidal, or in layers of two or more cells deep as stratified (layered), or ''compound'', either squamous, columnar or cuboidal. In some tissues, a layer of columnar cells may appear to be stratified due to the placement of the nuclei. This sort of tissue is called pseudostratified. All glands are made up of epithelia ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Reticular Lamina
Reticular fibers, reticular fibres or reticulin is a type of fiber in connective tissue composed of type III collagen secreted by reticular cells. Reticular fibers crosslink to form a fine meshwork (reticulin). This network acts as a supporting mesh in soft tissues such as liver, bone marrow, and the tissues and organs of the lymphatic system. History The term reticulin was coined in 1892 by M. Siegfried. Today, the term reticulin or reticular fiber is restricted to referring to fibers composed of type III collagen. However, during the pre-molecular era, there was confusion in the use of the term ''reticulin'', which was used to describe two structures: *the argyrophilic (silver staining) fibrous structures present in basement membranes *histologically similar fibers present in developing connective tissue. The history of the reticulin silver stain is reviewed by Puchtler ''et al.'' (1978). The abstract of this paper says: Maresch (1905) introduced Bielschowsky's silve ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Collagen III
Type III Collagen is a homotrimer, or a protein composed of three identical peptide chains (monomers), each called an alpha 1 chain of type III collagen. Formally, the monomers are called collagen type III, alpha-1 chain and in humans are encoded by the gene. Type III collagen is one of the fibrillar collagens whose proteins have a long, inflexible, triple-helical domain. Protein structure and function Type III collagen is synthesized by cells as a pre-procollagen. The signal peptide is cleaved off producing a procollagen molecule. Three identical type III procollagen chains come together at the carboxy-terminal ends, and the structure is stabilized by the formation of disulphide bonds. Each individual chain folds into left-handed helix and the three chains are then wrapped together into a right-handed superhelix, the triple helix. Prior to assembling the super-helix, each monomer is subjected to a number of post-translational modifications that occur while the monomer is ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Basement Membrane
The basement membrane is a thin, pliable sheet-like type of extracellular matrix that provides cell and tissue support and acts as a platform for complex signalling. The basement membrane sits between Epithelium, epithelial tissues including mesothelium and endothelium, and the underlying connective tissue. Structure As seen with the electron microscope, the basement membrane is composed of two layers, the basal lamina and the reticular lamina. The underlying connective tissue attaches to the basal lamina with collagen VII anchoring fibrils and fibrillin microfibrils. The basal lamina layer can further be subdivided into two layers based on their visual appearance in electron microscopy. The lighter-colored layer closer to the epithelium is called the lamina lucida, while the denser-colored layer closer to the connective tissue is called the lamina densa. The Electron microscope, electron-dense lamina densa layer is about 30–70 nanometers thick and consists of an underlying ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Epidermolysis Bullosa Dystrophica
Epidermolysis bullosa dystrophica or dystrophic EB (DEB) is an inherited disease affecting the skin and other organs. "Butterfly child" is the colloquial name for a child born with the disease, as their skin is seen to be as delicate and fragile as the wings of a butterfly. Signs and symptoms The deficiency in anchoring fibrils impairs the adherence between the epidermis and the underlying dermis. The skin of DEB patients is thus highly susceptible to severe blistering. Collagen VII is also associated with the epithelium of the esophageal lining, and DEB patients may have chronic scarring, webbing, and obstruction of the esophagus. Affected individuals are often severely malnourished due to trauma to the oral and esophageal mucosa and require feeding tubes for nutrition. They also have iron-deficiency anemia of uncertain origin, which leads to chronic fatigue. Open wounds on the skin heal slowly or not at all, often scarring extensively, and are particularly susceptible to infect ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
COL7A1
Collagen alpha-1(VII) chain is a protein that in humans is encoded by the ''COL7A1'' gene. It is composed of a triple helical, collagenous domain flanked by two non-collagenous domains, and functions as an anchoring fibril between the dermal-epidermal junction in the basement membrane. Mutations in COL7A1 cause all types of Dystrophic Epidermolysis Bullosa, dystrophic epidermolysis bullosa, and the exact mutations vary based on the specific type or subtype. It has been shown that interactions between the NC-1 domain of collagen VII and several other proteins, including Laminin, laminin-5 and Type IV collagen, collagen IV, contribute greatly to the overall stability of the basement membrane. Structure Type VII collagen is composed of three main domains in the following order: a non-collagenous domain, abbreviated NC-1; a collagenous domain; and a second non-collagenous domain, NC-2. The NC-1 domain has a cartilage matrix protein (CMP), nine Fibronectin type III domain, fibronectin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Epidermis (skin)
The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and hypodermis. The epidermis layer provides a barrier to infection from environmental pathogens and regulates the amount of water released from the body into the atmosphere through transepidermal water loss. The epidermis is composed of multiple layers of flattened cells that overlie a base layer (stratum basale) composed of columnar cells arranged perpendicularly. The layers of cells develop from stem cells in the basal layer. The human epidermis is a familiar example of epithelium, particularly a stratified squamous epithelium. The word epidermis is derived through Latin , itself and . Something related to or part of the epidermis is termed epidermal. Structure Cellular components The epidermis primarily consists of keratinocytes ( proliferating basal and differentiated suprabasal), which comprise 90% of its cells, but also contains melanocytes, Langerhans c ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Dermis
The dermis or corium is a layer of skin between the epidermis (with which it makes up the cutis) and subcutaneous tissues, that primarily consists of dense irregular connective tissue and cushions the body from stress and strain. It is divided into two layers, the superficial area adjacent to the epidermis called the papillary region and a deep thicker area known as the reticular dermis.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology'' (10th ed.). Saunders. Pages 1, 11–12. . The dermis is tightly connected to the epidermis through a basement membrane. Structural components of the dermis are collagen, elastic fibers, and extrafibrillar matrix.Marks, James G; Miller, Jeffery (2006). ''Lookingbill and Marks' Principles of Dermatology'' (4th ed.). Elsevier Inc. Page 8–9. . It also contains mechanoreceptors that provide the sense of touch and thermoreceptors that provide the sense of heat. In addition, hair follicles, ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lamina Propria
The lamina propria is a thin layer of connective tissue that forms part of the moist linings known as mucous membranes or mucosae, which line various tubes in the body, such as the respiratory tract, the gastrointestinal tract, and the urogenital tract. The lamina propria is a thin layer of loose (areolar) connective tissue, which lies beneath the epithelium, and together with the epithelium and basement membrane constitutes the mucosa. As its Latin name indicates, it is a characteristic component of the mucosa, or the mucosa's "own special layer." Thus, the term mucosa or mucous membrane refers to the combination of the epithelium and the lamina propria. The connective tissue of the lamina propria is loose and rich in cells. The cells of the lamina propria are variable and can include fibroblasts, lymphocytes, plasma cells, macrophages, eosinophilic leukocytes, and mast cells. It provides support and nutrition to the epithelium, as well as the means to bind to the underl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Connective Tissue
Connective tissue is one of the four primary types of animal tissue, along with epithelial tissue, muscle tissue, and nervous tissue. It develops from the mesenchyme derived from the mesoderm the middle embryonic germ layer. Connective tissue is found in between other tissues everywhere in the body, including the nervous system. The three meninges, membranes that envelop the brain and spinal cord are composed of connective tissue. Most types of connective tissue consists of three main components: elastic and collagen fibers, ground substance, and cells. Blood, and lymph are classed as specialized fluid connective tissues that do not contain fiber. All are immersed in the body water. The cells of connective tissue include fibroblasts, adipocytes, macrophages, mast cells and leucocytes. The term "connective tissue" (in German, ''Bindegewebe'') was introduced in 1830 by Johannes Peter Müller. The tissue was already recognized as a distinct class in the 18th century. ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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