[HTML][HTML] The Pro-α3 (V) Collagen Chain: complete primary structure, expression domains in adult and developing tissues, and comparison to the structures and …

Y Imamura, IC Scott, DS Greenspan - Journal of Biological Chemistry, 2000 - ASBMB
The low abundance fibrillar collagen type V is widely distributed in tissues as an α1 (V) 2 α2
(V) heterotrimer that helps regulate the diameters of fibrils of the abundant collagen type I.
Mutations in the α1 (V) and α2 (V) chain genes have been identified in some cases of
classical Ehlers-Danlos syndrome (EDS), in which aberrant collagen fibrils are associated
with connective tissue fragility, particularly in skin and joints. Type V collagen also exists as
an α1 (V) α2 (V) α3 (V) heterotrimer that has remained poorly characterized chiefly due to …