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...