title: Glomerular Collagen V Codeposition and Hepatic Perisinusoidal Collagen III Accumulation in Canine Collagen Type III Glomerulopathy.
abstract: Collagen type III glomerulopathy, also known as collagenofibrotic glomerulopathy, is a rare renal disease of unknown pathogenesis. The disease occurs in humans and animals and is characterized by massive glomerular accumulations of collagen type III. In the present study, we describe a Drever dog litter affected by an early onset variant of this glomerular disease, where 4 of 9 puppies developed renal failure within 50 days of age. Necropsy specimens of kidney from the 4 affected cases were studied by light microscopy, electron microscopy, and immunohistochemistry, and characteristic lesions compatible with a diagnosis of collagen type III glomerulopathy were found. In addition, 2 cases showed atypical epithelium in the collecting ducts of the medulla, so-called adenomatoid change. Immunohistochemistry of renal specimens from collagen type III glomerulopathy-affected dogs (n = 10) originating from two different dog strains, the Drever dogs and a mixed-breed strain, demonstrated that the deposited glomerular collagen is composed of a mixture of collagen III and collagen V. The distribution of the collagen V corresponded to the localization of collagen III; however, differences in staining intensity showed that collagen type III is the dominating component. Immunohistochemistry for collagen III (n = 9) and a transmission electron microscopic study (n = 1) showed hepatic perisinusoidal collagen type III deposition in affected cases from both dog strains. This is the first report documenting glomerular accumulations of collagen type V and perisinusoidal liver collagen III deposition in canine collagen type III glomerulopathy.
authors.affiliation: Faculty of Veterinary Medicine and Biosciences, Norwegian University of Life Sciences, Oslo/Ås, Norway Runa.Rortveit@nmbu.no.
authors.forename: R
authors.initials: R
authors.lastname: Rørtveit
authors.affiliation: Faculty of Veterinary Medicine and Biosciences, Norwegian University of Life Sciences, Oslo/Ås, Norway.
authors.forename: M R
authors.initials: MR
authors.lastname: Reiten
authors.affiliation: Faculty of Veterinary Medicine and Biosciences, Norwegian University of Life Sciences, Oslo/Ås, Norway.
authors.forename: F
authors.initials: F
authors.lastname: Lingaas
authors.affiliation: Enebakk Animal Clinic, Enebakk, Norway.
authors.forename: S B
authors.initials: SB
authors.lastname: Sveri
authors.affiliation: Centre for Cancer Biomedicine, Faculty of Medicine, University of Oslo, Oslo, Norway Institute for Cancer Research, The Norwegian Radium Hospital, Oslo University Hospital, Oslo, Norway.
authors.forename: A
authors.initials: A
authors.lastname: Brech
authors.affiliation: Faculty of Veterinary Medicine and Biosciences, Norwegian University of Life Sciences, Oslo/Ås, Norway.
authors.forename: A
authors.initials: A
authors.lastname: Espenes
authors.affiliation: Faculty of Veterinary Medicine and Biosciences, Norwegian University of Life Sciences, Oslo/Ås, Norway.
authors.forename: J H
authors.initials: JH
authors.lastname: Jansen
mesh_terms: D000818:Animals; D024061:Collagen Type III / Q000378:metabolism*; D024062:Collagen Type V / Q000378:metabolism*; D004283:Dog Diseases / Q000378:metabolism* / Q000473:pathology; D004285:Dogs; D005260:Female; D007150:Immunohistochemistry / Q000662:veterinary; D007674:Kidney Diseases / Q000378:metabolism / Q000473:pathology / Q000662:veterinary*; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron / Q000662:veterinary; D046529:Microscopy, Electron, Transmission / Q000662:veterinary
chemical_list: D024061:Collagen Type III; D024062:Collagen Type V
keywords: canine/dog; collagen; collagenofibrotic; glomerulopathy; hereditary; juvenile; kidney; nephropathy