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

A multi-layered quantitative in vivo expression atlas of the podocyte unravels kidney disease candidate genes

Rinschen, Markus M. ; Gödel, Markus ; Grahammer, Florian ; Zschiedrich, Stefan ; Helmstädter, Martin ; Kretz, Oliver ; Zarei, Mostafa ; Braun, Daniela A. ; Dittrich, Sebastian ; Pahmeyer, Caroline ; Schroder, Patricia ; Teetzen, Carolin ; Gee, HeonYung ; Daouk, Ghaleb ; Pohl, Martin ; Kuhn, Elisa ; Schermer, Bernhard ; Küttner, Victoria ; Boerries, Melanie ; Busch, Hauke ; Schiffer, Mario ; Bergmann, Carsten ; Krüger, Marcus ; Hildebrandt, Friedhelm ; Dengjel, Jörn ; Benzing, Thomas ; Huber, Tobias B.

In: Cell Reports, 2018, vol. 23, no. 8, p. 2495–2508

Damage to and loss of glomerular podocytes has been identified as the culprit lesion in progressive kidney diseases. Here, we combine mass spectrometry-based proteomics with mRNA sequencing, bioinformatics, and hypothesis-driven studies to provide a comprehensive and quantitative map of mammalian podocytes that identifies unanticipated signaling pathways. Comparison of the in vivo datasets...