title: Abnormal development of nephrons in claudin-16-defective Japanese black cattle.
abstract: The kidneys of 37 Japanese Black calves aged 2 to 65 months diagnosed with Claudin 16 (CL-16) defect by the DNA-based test were examined pathologically. The animals exhibited clinical symptoms such as growth impairment, renal failure, overgrowth of hooves, and anemia at a young age. There was no correlation between the time of onset and age. Kidney weights relative to body weight were similar to those in normal animals, but both kidney net weights and size were reduced due to atrophy in animals that showed severe renal dysfunction. Histopathological examination of the kidneys showed reduction in the number of glomeruli, compensatory hypertrophy of glomeruli and tubules, and glomerular and tubular atrophy accompanied by interstitial fibrosis and lymphocytic infiltration. Glomeruli were clearly less in number in the kidneys of CL-16-defective animals than those of normal animals even in the cases with mild lesions. A small number of immature glomeruli and tubules were also detected, suggesting that there were fewer nephrons developed at birth in CL-16-defective animals. It was suggested that a defect of the CL-16 gene is involved in the "abnormal development of nephrons". Immunohistopathological examination of the kidneys showed that the epithelium of thick ascending limb of Henle was stained with anti-CL-16 antibody in the control animals, but not in the affected animals, suggesting a defect of CL-16 in the epithelium of renal tubules in the affected animals.
authors.affiliation: Department of Veterinary Pathology, Faculty of Agriculture, Iwate University, 3-18-8 Ueda, Morioka Iwate 020-8550, Japan.
authors.forename: Kosuke
authors.initials: K
authors.lastname: Okada
authors.forename: Naoko
authors.initials: N
authors.lastname: Ishikawa
authors.forename: Kouichiro
authors.initials: K
authors.lastname: Fujimori
authors.forename: Masanobu
authors.initials: M
authors.lastname: Goryo
authors.forename: Manabu
authors.initials: M
authors.lastname: Ikeda
authors.forename: Jun
authors.initials: J
authors.lastname: Sasaki
authors.forename: Daisaku
authors.initials: D
authors.lastname: Watanabe
authors.forename: Akiko
authors.initials: A
authors.lastname: Takasuga
authors.forename: Takashi
authors.initials: T
authors.lastname: Hirano
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Sugimoto
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology / Q000662:veterinary*; D000818:Animals; D002417:Cattle; D002418:Cattle Diseases / Q000235:genetics / Q000473:pathology*; D057167:Claudins; D006130:Growth Disorders / Q000473:pathology / Q000662:veterinary*; D006652:Histological Techniques / Q000662:veterinary; D007150:Immunohistochemistry / Q000662:veterinary; D007564:Japan; D007678:Kidney Glomerulus / Q000473:pathology; D008565:Membrane Proteins / Q000235:genetics*; D009154:Mutation / Q000235:genetics; D009399:Nephrons / Q000002:abnormalities*; D009929:Organ Size; D051437:Renal Insufficiency / Q000473:pathology / Q000662:veterinary*
chemical_list: D057167:Claudins; D008565:Membrane Proteins; C502010:claudin 16