نتایج جستجو برای: type vii collagen

تعداد نتایج: 1402056  

Journal: :Human molecular genetics 2014
Teresa Odorisio Michela Di Salvio Angela Orecchia Giovanni Di Zenzo Eugenia Piccinni Francesca Cianfarani Antonella Travaglione Paolo Uva Barbara Bellei Andrea Conti Giovanna Zambruno Daniele Castiglia

Recessive dystrophic epidermolysis bullosa (RDEB) is a genodermatosis characterized by fragile skin forming blisters that heal invariably with scars. It is due to mutations in the COL7A1 gene encoding type VII collagen, the major component of anchoring fibrils connecting the cutaneous basement membrane to the dermis. Identical COL7A1 mutations often result in inter- and intra-familial disease v...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2018
Alexander Nyström Olivier Bornert Tobias Kühl Christine Gretzmeier Kerstin Thriene Jörn Dengjel Andrea Pfister-Wartha Dimitra Kiritsi Leena Bruckner-Tuderman

Genetic loss of collagen VII causes recessive dystrophic epidermolysis bullosa (RDEB), a skin fragility disorder that, unexpectedly, manifests also with elevated colonization of commensal bacteria and frequent wound infections. Here, we describe an unprecedented systemic function of collagen VII as a member of a unique innate immune-supporting multiprotein complex in spleen and lymph nodes. In ...

Journal: :Journal of Investigative Dermatology 2022

The healthy-looking non-lesional (NL) psoriatic skin already carries some alterations. These alterations include extracellular matrix defects such as the disruption of laminin networks, which may lead to impaired keratinocyte adhesion basal membrane. This process is associated with aberrant expression α5β1 integrin complex and extradomain-A-containing fibronectin (EDA+FN) around keratinocytes. ...

2013
Michael Roth Jun Zhong Celine Zumkeller Chong Teck S’ng Stephanie Goulet Michael Tamm

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 which IgE up-regulates cell proliferation and extracellular matrix deposition by human airway smooth muscle cells in asthma is unclear. OBJEC...

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