Fabry's disease: absence of storage as a feature of liver sinus endothelium.
Histochemical and electronoptic examination of 2 cases of Fabry's disease revealed no storage in the liver sinus endothelium while that of the portal vessels (artery, capillaries, and vein) and the central vein was affected by storage of a variable, though on average considerable degree. The findings are indirect evidence of the markedly different biology of the liver sinus endothelium within the capillary system.
D002196:Capillaries / Q000378:metabolism; D002554:Cerebrosides / Q000378:metabolism; D004727:Endothelium / Q000378:metabolism / Q000473:pathology; D000795:Fabry Disease / Q000378:metabolism* / Q000473:pathology; D006017:Glycolipids / Q000378:metabolism; D006651:Histocytochemistry; D006801:Humans; D008099:Liver / Q000098:blood supply / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008875:Middle Aged
D002554:Cerebrosides; D006017:Glycolipids