Anchoring fibrils (composed largely of type VII
collagen) extend from the
basal lamina of
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 intercell ...
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
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 ...
, 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
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-epide ...
, the gene that codes for a type of collagen 7.
See also
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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 rel ...
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Dermis
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Lamina propria
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Connective tissue
References
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External links
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{{Connective tissue
Structural proteins