2017 年 17 巻 p. 63-72
Brachymorphic mice have a chondrodystrophia induced by a point mutation in bifunctional sulfurylase kinase polypeptide functioning as ATP-sulfurylase and adenosine-phosphosulfate kinase. This disorder in the mice arises from the undersulfation of glycoconjugates in a numerous kind of organs and tissues. Unexpectedly, the aged female mouse (114 weeks old) with hematuria was found in brachymorphic mice. After the ultrastructural examination, pathological findings were identified in renal glomerular basement membrane. Consequently, attempts have been made to search the aging changes of ultrastructure in the renal glomerulus in aged normal and brachymorphic mice. In both aged normal and brachymorphic mice, humped intramembranous dense transformation and thickened glomerular basement membrane with multilayered matrix in lamina densa and narrowed lamina rara were shown in varying degrees. These pathological findings of glomerular basement membranes are markedly in brachymorphic mice than in normal mice. The disorders of renal glomerular basement membrane are thought to result from functional changing of aged glomerular basement membrane organizing cells (podocyte and endothelial cells) and undersulfated heparansulfate.
