[HTML][HTML] Inflammation-mediated fibroblast activation and immune dysregulation in collagen VII-deficient skin

M Anderson-Crannage, AM Ascensión… - Frontiers in …, 2023 - frontiersin.org
Inflammation is known to play a critical role in all stages of tumorigenesis; however, less is
known about how it predisposes the tissue microenvironment preceding tumor formation …

Transcriptomic profiling of recessive dystrophic epidermolysis bullosa wounded skin highlights drug repurposing opportunities to improve wound healing

A Onoufriadis, LE Proudfoot, C Ainali… - Experimental …, 2022 - Wiley Online Library
Chronic wounds present a major disease burden in people with recessive dystrophic
epidermolysis bullosa (RDEB), an inherited blistering skin disorder caused by mutations in …

Stromal microenvironment in type VII collagen-deficient skin: The ground for squamous cell carcinoma development

L Guerra, T Odorisio, G Zambruno, D Castiglia - Matrix Biology, 2017 - Elsevier
Recessive dystrophic epidermolysis bullosa (RDEB) is a skin fragility disease caused by
mutations that affect the function and/or the amount of type VII collagen (C7), the major …

[HTML][HTML] Combinatorial omics analysis reveals perturbed lysosomal homeostasis in collagen VII-deficient keratinocytes

K Thriene, BA Grüning, O Bornert, A Erxleben… - Molecular & Cellular …, 2018 - ASBMB
The extracellular matrix protein collagen VII is part of the microenvironment of stratified
epithelia and critical in organismal homeostasis. Mutations in the encoding gene COL7A1 …

[HTML][HTML] Mechanistic interrogation of mutation-independent disease modulators of RDEB identifies the small leucine-rich proteoglycan PRELP as a TGF-β antagonist …

E Chacón-Solano, C León, M Carretero, M Garcia… - Matrix Biology, 2022 - Elsevier
Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic extracellular matrix disease
caused by deficiency in type VII collagen (Col VII). The disease manifests with devastating …

Suppression of TGFβ and angiogenesis by type VII collagen in cutaneous SCC

VL Martins, MP Caley, K Moore… - Journal of the …, 2016 - academic.oup.com
Background: Individuals with severe generalized recessive dystrophic epidermolysis bullosa
(RDEB), an inherited blistering disorder caused by mutations in the COL7A1 gene, develop …

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

R Bernasconi, K Thriene… - EMBO Molecular …, 2021 - embopress.org
Recessive dystrophic epidermolysis bullosa (RDEB), a genetic skin blistering disease, is a
paradigmatic condition of tissue fragility‐driven multi‐organ fibrosis. Here, longitudinal …

[HTML][HTML] Collagen VII plays a dual role in wound healing

A Nyström, D Velati, VR Mittapalli… - The Journal of …, 2013 - Am Soc Clin Investig
Although a host of intracellular signals is known to contribute to wound healing, the role of
the cell microenvironment in tissue repair remains elusive. Here we employed 2 different …

Fibroblast-derived dermal matrix drives development of aggressive cutaneous squamous cell carcinoma in patients with recessive dystrophic epidermolysis bullosa

YZ Ng, C Pourreyron, JC Salas-Alanis, JHS Dayal… - Cancer research, 2012 - AACR
Patients with the genetic skin blistering disease recessive dystrophic epidermolysis bullosa
(RDEB) develop aggressive cutaneous squamous cell carcinoma (cSCC). Metastasis …

[HTML][HTML] A cancer stem cell-like phenotype is associated with miR-10b expression in aggressive squamous cell carcinomas

M Wimmer, R Zauner, M Ablinger… - Cell Communication and …, 2020 - Springer
Background Cutaneous squamous cell carcinomas (cSCC) are the primary cause of
premature deaths in patients suffering from the rare skin-fragility disorder recessive …