BMS 460 Lecture Notes - Hepatosplenomegaly, Multiple Myeloma, Arachnodactyly
26 Apr 2014
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Relatively few cells in large volume of extracellular matrix, ecm. Loose ct, in particular, houses blood vessels and nerves. The outer covering of blood vessels and nerves consists of a ct sheath. Collagen is a three-chain fibrous protein in which the chains coil around each other (called a coiled-coil structure) like the strands of a rope. This triple-helix molecular organization generates a protein with considerable tensile strength. A variety of cells produce more than 20 biochemically different types of collagen fibroblasts in the connective tissue and their equivalents the osteoblast (bone), chrondroblast (cartilage), and odontoblast (teeth) synthesize collagen. A mutation in col1a1 and col1a2 genes, encoding the 1 and 2 chains of type 1 collagen, respectively, involves cleavage sites for the n-terminal region of the molecule and interferes with the conversion of precollagen to collagen. This leads to defective cross-linking and a consequent reduction in the tensile strength of tendons (rich in type i collagen).
