Anchoring fibrils
Anchoring Fibrils
Anchoring fibrils are specialized structures in the extracellular matrix that play a crucial role in the attachment of the epidermis to the underlying dermis in the skin. These fibrils are primarily composed of type VII collagen, a unique type of collagen that is essential for maintaining the structural integrity of the skin.
Structure[edit | edit source]
Anchoring fibrils are composed of type VII collagen, which is synthesized by keratinocytes in the epidermis and fibroblasts in the dermis. Type VII collagen molecules assemble into dimers and then into anchoring fibrils, which are long, beaded structures that extend from the basement membrane zone into the papillary dermis. These fibrils form a network that interlinks with other components of the extracellular matrix, such as type I and type III collagen fibers, providing mechanical stability and resistance to shearing forces.
Function[edit | edit source]
The primary function of anchoring fibrils is to secure the epidermis to the dermis, thereby maintaining the structural cohesion of the skin. This attachment is crucial for the skin's ability to withstand mechanical stress and for the prevention of blistering. Anchoring fibrils achieve this by binding to laminin-332 (formerly known as laminin-5) in the lamina densa of the basement membrane and to type I and type III collagen in the dermis.
Clinical Significance[edit | edit source]
Defects in anchoring fibrils, particularly those caused by mutations in the COL7A1 gene that encodes type VII collagen, can lead to a group of genetic disorders known as dystrophic epidermolysis bullosa (DEB). DEB is characterized by fragile skin that blisters easily in response to minor trauma. The severity of the condition can vary widely, from mild blistering to severe, life-threatening forms.
Research and Developments[edit | edit source]
Recent advances in gene therapy and protein replacement therapy are being explored as potential treatments for conditions caused by defects in anchoring fibrils. These therapies aim to restore the function of type VII collagen and improve the structural integrity of the skin.
Also see[edit | edit source]
| Collagen | |
|---|---|
 |
|
| Overview | |
| Type | Protein |
| Function | Structural component of connective tissues |
| Location | Skin, tendons, ligaments, cartilage, bone, blood vessels |
| Types | |
| Related Topics | |
| See Also | |
Dermatology and Rheumatologic diseases A - Z
A | B | C | E | F | G | H | I | J | K | L | M | O | P | R | S | T | V
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