title: Animal model: renal lesions in cats with Chediak-Higashi-Steinbrinck syndrome.
abstract: In this study designed to characterize the renal lesions in cats with the autosomal recessive Chediak-Higashi-Steinbrinck syndrome (CHS), the renal tubular epithelial cells of CHS cats were examined by light microscopy. Numerous cells of proximal convoluted and straight tubules, thin loop of Henle, distal convoluted and straight tubules, and collecting tubules contained enlarged cytoplasmic granules morphologically consistent with lysosomes. In general, the enlarged lysosomes were larger and more numerous in proximal convoluted and straight tubular cells and were generally more massive in older cats. The lesions observed were similar to those in the renal epithelial cells of other species with CHS and were consistent with those reported previously in other tissues of CHS cats. It is concluded that CHS cats are an appropriate model in which to study the effects of the CHS renal lesions on renal function in this syndrome.
mesh_terms: D000818:Animals; D002415:Cats; D002609:Chediak-Higashi Syndrome / Q000235:genetics / Q000473:pathology*; D004195:Disease Models, Animal; D004848:Epithelium / Q000473:pathology; D005260:Female; D007668:Kidney / Q000473:pathology*; D007684:Kidney Tubules / Q000473:pathology; D008247:Lysosomes / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron