Vascular pathology in galactosialidosis.
This article immunohistochemically and ultrastructurally examines a brain with galactosialidosis, focusing on the structure of blood vessel endothelium. Lysosomes were observed in the expanded cytoplasm of the endothelial cells, which were vacuolated by light microscopy. Immunoreactivity for CD31, one of the vascular cell adhesion molecules, was minimal to faint in endothelial cells with vacuolations. The loss of CD31 immunoreactivity and breakdown of vascular cell adhesion molecules in vacuolated endothelial cells seem to promote the development of brain infarctions. Moreover, diffuse and various degrees of axonal damage, most likely caused by vascular disorder in cerebral or cerebellar white matter, was reported using the amyloid precursor protein (APP) immunohistochemical method.
Arai
Y
Y
Department of Clinical Laboratory, National Center Hospital for Mental, Nervous and Muscular Disorders, Kodaira, Tokyo, Japan.
Edwards
V
V
Takashima
S
S
Becker
L E
LE
D016564:Amyloid beta-Protein Precursor / Q000378:metabolism; D001369:Axons / Q000378:metabolism / Q000648:ultrastructure; D001921:Brain / Q000098:blood supply*; D001927:Brain Diseases / Q000201:enzymology / Q000473:pathology*; D004730:Endothelium, Vascular / Q000378:metabolism / Q000648:ultrastructure*; D017809:Fatal Outcome; D006801:Humans; D007124:Immunoenzyme Techniques; D007231:Infant, Newborn; D016464:Lysosomal Storage Diseases / Q000201:enzymology / Q000473:pathology*; D008247:Lysosomes / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D009439:Neuraminidase / Q000172:deficiency*; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000378:metabolism; D001616:beta-Galactosidase / Q000172:deficiency*
D016564:Amyloid beta-Protein Precursor; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D009439:Neuraminidase; D001616:beta-Galactosidase