[HTML][HTML] Vascular Ehlers-Danlos syndrome mutations in type III collagen differently stall the triple helical folding

K Mizuno, S Boudko, HP Bächinger - Journal of Biological Chemistry, 2013 - ASBMB
Vascular Ehlers-Danlos syndrome (EDS) type IV is the most severe form of EDS. In many
cases the disease is caused by a point mutation of Gly in type III collagen. A slower folding of
the collagen helix is a potential cause for over-modifications. However, little is known about
the rate of folding of type III collagen in patients with EDS. To understand the molecular
mechanism of the effect of mutations, a system was developed for bacterial production of
homotrimeric model polypeptides. The C-terminal quarter, 252 residues, of the natural …