Type III collagen is the second most abundant collagen in tissues and is found most commonly in tissues exhibiting elastic properties such as skin, lungs, intestinal walls and walls of blood vessels. It is a homotrimer comprised of three alpha-1 chains and resembles other fibrillar collagens in structure and function. It is synthesized as procollagen, similary to collagen I, but the N-terminal propeptide remains attached in the mature fibrillar type III form.

Mutations of type III collagen causes Ehlers-Danlos syndrome, EDS IV, which affect arteries, internal organs, joints and skin, and may cause sudden death when the large arteries rupture.

Symbols/Related Terms:

• COL3A1
• Collagen type III, alpha 1
• Collagen III, alpha-1 polypeptide
• collagen, type III, alpha 1 (Ehlers-Danlos syndrome type IV, autosomal dominant)
• EDS4A