title: Contraction of collagen lattices by fibroblasts from patients and animals with heritable disorders of connective tissue.
abstract: Fibroblasts derived from patients and animals presenting various heritable connective tissue disorders were investigated for the ability to retract a reconstituted collagen matrix. When seeded into gels, dermatosparactic calf and sheep fibroblasts did not exhibit the elongated shape of normal fibroblasts and did not contract the collagen lattice to the same extent as control fibroblasts. In contrast, several cell strains obtained from patients with Ehlers-Danlos syndrome type VII displayed contractile properties for collagen gels similar to controls. Delayed contraction was noted by two strains of fibroblasts from patients with Ehlers-Danlos syndrome type IV, whereas fibroblasts from patients with osteogenesis imperfecta, Marfan syndrome and cutis laxa had normal retraction properties.
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D002417:Cattle; D002478:Cells, Cultured; D002648:Child; D003094:Collagen / Q000502:physiology*; D003095:Collagen Diseases / Q000503:physiopathology*; D003483:Cutis Laxa / Q000503:physiopathology; D004535:Ehlers-Danlos Syndrome / Q000503:physiopathology; D005109:Extracellular Matrix / Q000502:physiology*; D005347:Fibroblasts / Q000502:physiology*; D006801:Humans; D008382:Marfan Syndrome / Q000503:physiopathology; D010013:Osteogenesis Imperfecta / Q000503:physiopathology; D012756:Sheep
chemical_list: D003094:Collagen