Enzyme replacement in nervous tissue after allogeneic bone-marrow transplantation for fucosidosis in dogs.
Bone-marrow transplantation after total lymphoid irradiation in an alpha-L-fucosidase-deficient dog raised the enzyme activity in both visceral and neural tissues with consequent reduction in the severity of storage lesions. These results offer hope that early marrow transplantation may prevent the development of disease in neurovisceral storage disorders.
D000818:Animals; D016026:Bone Marrow Transplantation; D004195:Disease Models, Animal; D004285:Dogs; D005260:Female; D005645:Fucosidosis / Q000628:therapy*; D008221:Lymphoid Tissue / Q000528:radiation effects; D008297:Male; D009417:Nerve Tissue / Q000201:enzymology*; D013997:Time Factors; D014184:Transplantation, Homologous; D005644:alpha-L-Fucosidase / Q000032:analysis*
D005644:alpha-L-Fucosidase