Article
Loss of collagen VII is associated with reduced transglutaminase 2 abundance and activity.
The Journal of investigative dermatology - 1 Sept 2014
Küttner Victoria, Mack Claudia, Gretzmeier Christine, Bruckner-Tuderman Leena, Dengjel Jörn
Abstract excerpt
Absence of collagen VII leads to widespread cellular and tissue phenotypes. However, the underlying molecular mechanisms are not well understood. To gain insights into cellular responses to loss of collagen VII, we undertook a quantitative disease proteomics approach. By using recessive dystrophic epidermolysis bullosa (RDEB), a skin blistering disease caused by collagen VII deficiency, as a genetic model,...
Topics
- Autophagy
- Cell Adhesion
- Collagen Type VII
- Dermis
- Enzyme Activation
- Epidermal Cells
- Epidermis
- Epidermolysis Bullosa Dystrophica
- Fibroblasts
- GTP-Binding Proteins
- Humans
- Infant, Newborn
- Phenotype
- Primary Cell Culture
- Protein Glutamine gamma Glutamyltransferase 2
- Proteome
- Transglutaminases