title: Collagen VII expression in glomerular sclerosis.
abstract: Glomerular sclerosis is the final stage of a variety of kidney diseases and matrix molecules not normally expressed in the extracellular matrix are synthesized and accumulate during the sclerotic process. Collagen type VII is the major component of the anchoring fibrils at the dermal-epidermal junction, but it is usually not present in normal glomeruli. The aim of this study was to investigate whether this type of fibrillary collagen, different from types I and III, is expressed in chronically diseased glomerular extracellular matrix. The presence and distribution of collagen VII have been examined in 50 renal biopsies by indirect immunofluorescence staining, standard electron microscopy, and immuno-electron microscopy. In selected cases, collagen VII mRNA expression was also measured by RT-PCR on isolated glomeruli. Cases included focal segmental glomerulosclerosis, minimal change disease, membranous glomerulonephritis, IgA nephropathy, SLE nephritis, diabetic glomerulosclerosis, ischaemic renal disease, extracapillary glomerulonephritis, and end-stage renal disease. Collagen VII protein and mRNA expression was absent or present in trace amounts in normal kidneys or in disorders with only a mild increase of mesangial matrix, without scarring of the tuft. Maximal expression was evident in the presence of adhesions between the glomerular tuft and Bowman's capsule or fibrous crescents. The results showed that collagen VII is actively synthesized and laid down in areas of glomerular and/or tubular scarring, irrespective of the underlying disease, confirming the de novo expression of fibrillary collagens in diseased renal extracellular matrix. The appearance of an anchoring collagen may be a response to support mechanical stress and it takes part in the process of cell proliferation and tissue repair.
authors.affiliation: Dipartimento di Medicina Sperimentale e Patologia, Università 'La Sapienza', Roma, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Onetti Muda
authors.forename: L
authors.initials: L
authors.lastname: Ruzzi
authors.forename: S
authors.initials: S
authors.lastname: Bernardini
authors.forename: A
authors.initials: A
authors.lastname: Teti
authors.forename: T
authors.initials: T
authors.lastname: Faraggiana
mesh_terms: D016022:Case-Control Studies; D024143:Collagen Type VII / Q000032:analysis* / Q000235:genetics; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007674:Kidney Diseases / Q000378:metabolism; D007678:Kidney Glomerulus / Q000737:chemistry* / Q000473:pathology*; D008099:Liver / Q000737:chemistry; D008854:Microscopy, Electron; D016253:Microscopy, Immunoelectron; D012333:RNA, Messenger / Q000032:analysis; D012598:Sclerosis; D012867:Skin / Q000737:chemistry
chemical_list: D024143:Collagen Type VII; D012333:RNA, Messenger