Figure 4.

Type I Collagen Studies

(A) Intracellular collagen folding in proband fibroblasts is moderately delayed. n = 3 for each cell line. ^∗ = p < 0.05.

(B) Compared to controls, collagen biochemistry of proband fibroblasts showed broadened bands and delayed migration of the α1(I)- and α2(I)-collagen chains.

(C) Collagen secretion assay. Left panel shows SDS-PAGE analysis of the intracellular and secreted fibrillar types I and V collagen during a 180 min time range (time points are indicated above the gel). Right panel shows measurement of the intensities of the type I collagen bands (ImageJ). This revealed that type I collagen is more slowly secreted from the proband fibroblasts. n = 2–3 for each cell line. ^∗p < 0.05.