Université de Fribourg

Proteomic profiling of fibroblasts isolated from chronic wounds identifies disease-relevant signaling pathways

Berberich, Bettina ; Thriene, Kerstin ; Gretzmeier, Christine ; Kühl, Tobias ; Bayer, Hans ; Athanasiou, Ioannis ; Rafei-Shamsabadi, David Ali ; Bruckner-Tuderman, Leena ; Nyström, Alexander ; Kiritsi, Dimitra ; Dengjel, Jörn

In: Journal of Investigative Dermatology, 2020, vol. 140, no. 11, p. 2280-2290.e4

Chronic skin wounds accompany many prevalent age-related diseases and are a major cause of morbidity and mortality. Both keratinocytes and fibroblasts contribute to the pathomechanisms in chronic skin wounds. Dysregulated pathways in the epidermis have been extensively studied, but little is known of the influence of dermal fibroblasts on chronic wounding. We isolated fibroblasts from chronic...

Université de Fribourg

Constitutional absence of epithelial integrin α3 impacts the composition of the cellular microenvironment of ILNEB keratinocytes

He, Yinghong ; Thriene, Kerstin ; Boerries, Melanie ; Hausser, Ingrid ; Franzke, Claus-Werner ; Busch, Hauke ; Dengjel, Jörn ; Has, Cristina

In: Matrix Biology, 2018, vol. 74, p. 62-76

Integrin α3β1, a major epidermal adhesion receptor is critical for organization of the basement membrane during development and wound healing. Integrin α3 deficiency leads to interstitial lung disease, nephrotic syndrome and epidermolysis bullosa (ILNEB), an autosomal recessive multiorgan disease characterized by basement membrane abnormalities in skin, lung and kidney. The pathogenetic...

Université de Fribourg

Impaired lymphoid extracellular matrix impedes antibacterial immunity in epidermolysis bullosa

Nyström, Alexander ; Bornert, Olivier ; Kühl, Tobias ; Gretzmeier, Christine ; Thriene, Kerstin ; Dengjel, Jörn ; Pfister-Wartha, Andrea ; Kiritsi, Dimitra ; Bruckner-Tuderman, Leena

In: Proceedings of the National Academy of Sciences, 2018, vol. 115, no. 4, p. E705–E714

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....

Université de Fribourg

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

Thriene, Kerstin ; Grüning, Björn Andreas ; Bornert, Olivier ; Erxleben, Anika ; Leppert, Juna ; Athanasiou, Ioannis ; Weber, Ekkehard ; Kiritsi, Dimitra ; Nyström, Alexander ; Reinheckel, Thomas ; Backofen, Rolf ; Has, Cristina ; Bruckner-Tuderman, Leena ; Dengjel, Jörn

In: Molecular & Cellular Proteomics, 2018, vol. 17, no. 4, p. 565-579

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 lead to the skin disorder dystrophic epidermolysis bullosa (DEB), are linked to skin fragility and progressive inflammation-driven fibrosis that facilitates aggressive skin cancer. So far, these changes have been...