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Collagen VI, a microfibrillar protein found in virtually all connective tissues, is composed of three distinct subunits, alpha 1(VI), alpha 2(VI), and alpha 3(VI), which associate intracellularly to form triple helical heterotrimeric monomers then dimers and tetramers. The secreted tetramers associate end-to-end to form beaded microfibrils. Although the basic steps in assembly and the structure of