Electron microscopic investigation of inclusion material in a case of adult metachromatic leukodystrophy; observations on kidney biopsy, peripheral nerve and cerebral white matter.
The fine structural characteristics of storage products in peripheral nerve, kidney and cerebral white matter, from a case of adult metachromatic leukodystrophy are described. There were pronounced differences from the fine structural aspects in late infantile cases. A large proportion of the inclusions did not exhibit a unit membrane. An hypothesis is proposed to clarify the delayed manifestation of this type of metachromatic leukodystrophy until childhood.
D000135:Acid Phosphatase / Q000032:analysis; D000328:Adult; D001921:Brain / Q000648:ultrastructure*; D003711:Demyelinating Diseases; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D007684:Kidney Tubules / Q000648:ultrastructure*; D007686:Kidney Tubules, Distal / Q000648:ultrastructure*; D007966:Leukodystrophy, Metachromatic / Q000473:pathology*; D008247:Lysosomes; D008264:Macrophages / Q000648:ultrastructure; D008297:Male; D009474:Neurons / Q000648:ultrastructure; D010525:Peripheral Nerves / Q000201:enzymology / Q000648:ultrastructure*; D012583:Schwann Cells / Q000648:ultrastructure; D013433:Sulfoglycosphingolipids / Q000378:metabolism
D013433:Sulfoglycosphingolipids; D000135:Acid Phosphatase