Results 31 to 40 of about 23,288 (156)

Pro‐inflammatory immunity supports fibrosis advancement in epidermolysis bullosa: intervention with Ang‐(1‐7)

EMBO Molecular Medicine, 2021
Recessive dystrophic epidermolysis bullosa (RDEB), a genetic skin blistering disease, is a paradigmatic condition of tissue fragility‐driven multi‐organ fibrosis.
Rocco Bernasconi, Kerstin Thriene, Elena Romero‐Fernández, Christine Gretzmeier, Tobias Kühl, Mareike Maler, Pauline Nauroy, Svenja Kleiser, Anne‐Catherine Rühl‐Muth, Michael Stumpe, Dimitra Kiritsi, Stefan F Martin, Boris Hinz, Leena Bruckner‐Tuderman, Jörn Dengjel, Alexander Nyström   +15 more
doaj   +1 more source

Structural and biophysical characterization of the type VII collagen vWFA2 subdomain leads to identification of two binding sites

FEBS Open Bio, 2020
Type VII collagen is an extracellular matrix protein, which is important for skin stability; however, detailed information at the molecular level is scarce.
Jan M. Gebauer, Florian Flachsenberg, Cordula Windler, Barbara Richer, Ulrich Baumann, Karsten Seeger   +5 more
doaj   +1 more source

Epidermolysis Bullosa (EB) Acquisita in an Adult Patient with Previously Unrecognized Mild Dystrophic EB and Biallelic COL7A1 Mutations

Acta Dermato-Venereologica, 2018
Circulating anti-type VII collagen autoantibodies are frequently detected in patients with recessive dystrophic epidermolysis bullosa (RDEB). However, evidence supporting their pathogenic role in inducing epidermolysis bullosa acquisita (EBA) has been ...
Liliana Guerra, Angelo Giuseppe Condorelli, Paola Fortugno, Valentina Calabresi, Cristina Pedicelli, Giovanni Di Zenzo, Daniele Castiglia   +6 more
doaj   +1 more source

Immunohistochemical expression of collagen type VII antibody in the human fetal temporomandibular joint discs [PDF]

Einstein (São Paulo), 2007
Objective: To study the articular disc morphology and to analyze theimmunohistochemical expression of the type VII collagen marker inhuman fetal temporomandibular joint discs at different gestational ages.Methods: Twenty human fetal temporomandibular ...
Luís Otávio Carvalho de Morais, Fábio Redivo Lodi, Sergio Ricardo Marques, Celina Tizuko Fijiyama Oshima, João Antão Fernandes Júnior, Ricardo Luiz Smith, Luís Garcia Alonso   +6 more
doaj  

Deficient skin proteins rescue of expression in patients with epidermolysis bullosa: efficacy of gentamicin [PDF]

Vestnik Dermatologii i Venerologii
Epidermolysis bullosa is a group of rare hereditary skin diseases based on mutations in the genes of structural proteins of the epidermis and the dermal-epidermal junction.
Olga G. Artamonova, Arfenya E. Karamova, Alexey A. Kubanov, Vadim V. Chikin, Ekaterina S. Monchakovskaya   +4 more
doaj   +1 more source

Rat model for dominant dystrophic epidermolysis bullosa: glycine substitution reduces collagen VII stability and shows gene-dosage effect. [PDF]

PLoS ONE, 2013
Dystrophic epidermolysis bullosa, a severely disabling hereditary skin fragility disorder, is caused by mutations in the gene coding for collagen VII, a specialized adhesion component of the dermal-epidermal junction zone.
Alexander Nyström, Jens Buttgereit, Michael Bader, Tatiana Shmidt, Cemil Ozcelik, Ingrid Hausser, Leena Bruckner-Tuderman, Johannes S Kern   +7 more
doaj   +1 more source

Preclinical model for phenotypic correction of dystrophic epidermolysis bullosa by in vivo CRISPR-Cas9 delivery using adenoviral vectors

Molecular Therapy: Methods & Clinical Development, 2022
Recessive dystrophic epidermolysis bullosa, a devastating skin fragility disease characterized by recurrent skin blistering, scarring, and a high risk of developing squamous cell carcinoma is caused by mutations in COL7A1, the gene encoding type VII ...
Marta García, Jose Bonafont, Jesús Martínez-Palacios, Rudan Xu, Giandomenico Turchiano, Stina Svensson, Adrian J. Thrasher, Fernando Larcher, Marcela Del Rio, Rubén Hernández-Alcoceba, Marina I. Garín, Ángeles Mencía, Rodolfo Murillas   +12 more
doaj   +1 more source

The role of IgE-receptors in IgE-dependent airway smooth muscle cell remodelling. [PDF]

PLoS ONE, 2013
BACKGROUND: In allergic asthma, IgE increases airway remodelling but the mechanism is incompletely understood. Airway remodelling consists of two independent events increased cell numbers and enhanced extracellular matrix deposition, and the mechanism by
Michael Roth, Jun Zhong, Celine Zumkeller, Chong Teck S'ng, Stephanie Goulet, Michael Tamm   +5 more
doaj   +1 more source

Deletion of a Pathogenic Mutation-Containing Exon of COL7A1 Allows Clonal Gene Editing Correction of RDEB Patient Epidermal Stem Cells

Molecular Therapy: Nucleic Acids, 2018
Recessive dystrophic epidermolysis bullosa is a severe skin fragility disease caused by loss of functional type VII collagen at the dermal-epidermal junction. A frameshift mutation in exon 80 of COL7A1 gene, c.6527insC, is highly prevalent in the Spanish
Ángeles Mencía, Cristina Chamorro, Jose Bonafont, Blanca Duarte, Almudena Holguin, Nuria Illera, Sara G. Llames, Maria José Escámez, Ingrid Hausser, Marcela Del Río, Fernando Larcher, Rodolfo Murillas   +11 more
doaj   +1 more source

Adjuvant polyarthritis. vii. The role of type ii collagen in pathogenesis [PDF]

Arthritis & Rheumatism, 1982
AbstractThe development of immune response in rats directed toward EL4 cells, after the injection of EL4 cells suspended in a saline/oil emulsion, was enhanced by the incorporation of Mycobacterium into the saline/oil emulsion; the incorporation of type II collagen into the saline–acetic acid/oil emulsion in concentrations ranging from 0.5–25 μg/ml had
Y, Iizuka, Y H, Chang
openaire   +2 more sources