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This condition is caused by a genetic defect coding for anchoring fibrils.
Anchoring fibrils are essential to the functional integrity of the dermoepidermal junction.
Bart syndrome is caused by ultrastructural abnormalities in the anchoring fibrils.
"Type VII collagen forms an extended network of anchoring fibrils."
"Epithelial origin of cutaneous anchoring fibrils."
The deficiency in anchoring fibrils impairs the adherence between the epidermis and the underlying dermis.
Bruckner-Tuderman L: Hereditary skin diseases of anchoring fibrils.
Anchoring fibrils are thought to form a structural link between the epidermal basement membrane and the fibrillar collagens in the upper dermis.
Burgeson, R. E. "Type VII collagen, anchoring fibrils, and epidermolysis bullosa."
Beneath the lamina densa, loop-structured, cross-banded anchoring fibrils extend more than 300 nm beneath the basement membrane within the papillary dermis.
Together with the anchoring fibrils and anchoring filaments, these are collectively termed the HD-stable adhesion complex or HD-anchoring filament complex.
The lamina reticularis is attached to the basal lamina with anchoring fibrils (type VII collagen fibers) and microfibrils (fibrillin).
Anchoring fibrils composed of type VII collagen extend from the basal lamina into the underlying reticular lamina and loop around collagen bundles.
Epidermolysis bullosa dystrophica, also known as Dystrophic EB (DEB) is a chronic skin condition caused when anchoring fibrils are abnormal, diminished, or absent.
Basal laminae are attached to reticular fibers made of type III collagen in the underlying connective tissues by anchoring fibrils of type VII collagen.
McGrath J. A., Ishida-Yamamoto A., O'Grady A., Leigh I. M., Eady R. A. J. "Structural variations in anchoring fibrils in dystrophic epidermolysis bullosa: correlation with type VII collagen expression."
The basal cells in the stratum basale of the epidermis connect to the basement membrane by the anchoring filaments of hemidesmosomes; the cells of the papillary layer of the dermis are attached to the basement membrane by anchoring fibrils, which consist of type VII collagen.