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Molecular cloning and characterization of type VII collagen cDNA

Biochemical and Biophysical Research Communications, 1992
Type VII collagen, located in human epidermal basement membrane, is the primary pathogenic target molecule in epidermolysis bullosa acquisita and epidermolysis bullosa dystrophica. Using a monoclonal antibody against the non-collagenous domain of type VII collagen, approximately 1 Kb cDNA was isolated from human keratinocyte library.
T, Tanaka   +3 more
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Distribution of type-VII collagen in xenografted human carcinomas

Cell & Tissue Research, 1993
The distribution of type-VII collagen, the main molecular component of the anchoring fibrils (AF) attaching the basal lamina (BL, lamina densa of the basement membrane) to the surrounding connective tissue, was investigated in four xenografted human carcinomas of the hypopharynx (H-Stg 1), the lung (L 261), the sigmoid colon (CA 1), and the rectum (R ...
P, Köpf-Maier, C, Schröter-Kermani
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Abnormal deposition of type VII collagen in Kindler syndrome

Archives of Dermatological Research, 1999
Kindler syndrome is an extremely rare genetic disorder with features of epidermolysis bullosa and poikiloderma congenitale. Approximately 70 cases have been documented in the past 50 years, but only a few investigations of the basement membrane components have been done on these patients.
C B, Wiebe, H S, Larjava
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Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

Clinical Reviews in Allergy & Immunology, 2007
Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune, mechanobullous disease with clinical features reminiscent of genetic dystrophic epidermolysis bullosa (DEB). EBA patients have skin fragility, blisters, scars, and milia formation. DEB is due to a genetic defect in the gene-encoding type VII collagen, which makes anchoring fibrils ...
David T, Woodley   +2 more
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The epitope for anti-type VII collagen monoclonal antibody (LH7:2) locates at the central region of the N-terminal non-collagenous domain of type VII collagen

British Journal of Dermatology, 2006
The monoclonal antibody LH7:2, which recognizes type VII collagen, is now used in the diagnosis and prenatal diagnosis of epidermolysis bullosa dystrophica. We constructed the expression vector which contains the cDNA fragment of type VII collagen. Western blot with LH7:2 was carried out with the resultant fusion proteins which overlap each other, and ...
T, Tanaka   +3 more
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Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita

2008
Epidermolysis bullosa acquisita (EBA) is an acquired, mechanobullous disease characterized by autoimmunity to type VII collagen. Type VII collagen makes anchoring fibrils, structures that connect the epidermis and its underlying basement membrane zone to the papillary dermis.
Jennifer, Remington   +3 more
openaire   +2 more sources

Type VII collagen is a component of cylindroma basement membrane zone

Journal of Cutaneous Pathology, 1989
Cylindroma basement membranes (BMs) were studied by electron microscopy and immunofluorescence with antibodies to Types IV and VII collagen in order to correlate the ultrastructure with the biochemical characteristics. Type IV collagen served as a marker for lamina densa structures and Type VII collagen for the anchoring fibrils. Ultrastructurally, the
Pfaltz M   +2 more
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Autoimmunity to type VII collagen.

Seminars in dermatology, 1991
The epidermolysis bullosa acquisita (EBA) antigen is type VII collagen that is found within the anchoring fibrils of the basement membrane zone beneath stratified epithelia. Autoimmunity to the EBA antigen/type VII collagen has been associated with three diseases: EBA, bullous systemic lupus erythematosus (SLE) and a subset of linear IgA bullous ...
D T, Woodley, Y, Sarret, R A, Briggaman
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Type VII Collagen Disorders Simplified

Cutis
Epidermolysis bullosa acquisita (EBA) and bullous systemic lupus erythematosus (BSLE) are autoimmune mechanobullous diseases that are caused by autoantibodies directed against type VII collagen. The functionality of type VII collagen is vital to the skin and mucous membranes because it makes up the anchoring fibrils that adhere the epithelium to the ...
openaire   +2 more sources

Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa

Nature Medicine, 2004
Dystrophic epidermolysis bullosa (DEB) is a family of inherited mechano-bullous disorders that are caused by mutations in the type VII collagen gene and for which ex vivo gene therapy has been considered. To develop a simpler approach for treating DEB, we evaluated the feasibility of protein-based therapy by intradermally injecting human recombinant ...
David T, Woodley   +6 more
openaire   +2 more sources

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