Molecular pathobiology of human collagens.
The fibril-forming collagens (types I-III, V and XI) represent a homogeneous and evolutionary related group of proteins and genes. In addition to serving as supportive elements, these macromolecules influence the spatial and ontogenic diversity of extracellular matrices, for they regulate a number of developmental programs and cellular activities, such as adhesion, proliferation and migration. Deranged expression of fibrillar collagen genes results in a number of inherited and acquired disorders which greatly affect the structural integrity of the organism. An understanding of collagen biosynthesis and regulation in normal and diseased states provides an opportunity to dissect biological problems which relate to a wide variety of subjects, including morphogenesis, relationships between structure and function of proteins, gene expression and human mutations.
Ramirez
F
F
Department of Microbiology-Immunology, Morse Institute of Molecular Genetics, State University of New York, Brooklyn 11203.
Boast
S
S
D'Alessio
M
M
Prince
J
J
Su
M W
MW
Vissing
H
H
D000818:Animals; D003094:Collagen / Q000096:biosynthesis / Q000235:genetics*; D003095:Collagen Diseases / Q000235:genetics / Q000378:metabolism; D006801:Humans
D003094:Collagen