title: Active collagen synthesis by pulmonary arteries in human primary pulmonary hypertension.
abstract: Immunohistochemistry was performed on lung tissue obtained from patients with severe unexplained pulmonary hypertension using an antibody to the amino-terminal end of the procollagen type I propeptide. This antibody identifies newly synthesized alpha I(I) procollagen before cleavage of the amino-terminal propeptide following secretion and, therefore, can identify sites of active collagen deposition. Procollagen was detected in the media, media and neointima, or neointima alone of a large number of small muscular arteries from hypertensive lungs. Normal adult lungs were negative. Neointimal cells in remodeled small muscular arteries stained positively for alpha-smooth muscle actin and desmin consistent with a smooth muscle lineage. These data suggest smooth muscle-like cells in small muscular arteries are actively synthesizing collagen in patients with severe unexplained pulmonary hypertension.
authors.affiliation: Department of Medicine, Jewish Hospital, Washington University Medical Center, St. Louis, MO 63110.
authors.forename: M D
authors.initials: MD
authors.lastname: Botney
authors.forename: M J
authors.initials: MJ
authors.lastname: Liptay
authors.forename: L R
authors.initials: LR
authors.lastname: Kaiser
authors.forename: J D
authors.initials: JD
authors.lastname: Cooper
authors.forename: W C
authors.initials: WC
authors.lastname: Parks
authors.forename: R P
authors.initials: RP
authors.lastname: Mecham
mesh_terms: D000199:Actins / Q000276:immunology; D000911:Antibodies, Monoclonal; D003094:Collagen / Q000096:biosynthesis*; D003893:Desmin / Q000276:immunology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000378:metabolism* / Q000473:pathology; D007150:Immunohistochemistry; D009131:Muscle, Smooth, Vascular / Q000166:cytology; D011347:Procollagen / Q000276:immunology; D011651:Pulmonary Artery / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology; D013194:Staining and Labeling
chemical_list: D000199:Actins; D000911:Antibodies, Monoclonal; D003893:Desmin; D011347:Procollagen; D003094:Collagen