A freeze-fracture study on GM2 gangliosidosis.
Membranous cytoplasmic bodies (MCBs) in neurons from an autopsy case with Tay-Sachs disease were investigated using the freeze-fracture technique. Despite formalin fixation for months, the structures of storage material were well preserved and showed complex and compound MCBs with typical arrangement of lamellae. Each lamella consisted of a clear space of about 27 nm delimited on both sides by a small fracture line similar to plasma membranes as seen in electron micrographs of freeze-fracture studies. Our investigation shows that freeze-fracturing of autopsy material is a valuable method and can be performed on neuronal storage material.
Volk
B
B
D002467:Cell Nucleus / Q000648:ultrastructure; D002675:Child, Preschool; D003593:Cytoplasm / Q000648:ultrastructure; D005260:Female; D005614:Freeze Fracturing; D006801:Humans; D007425:Intracellular Membranes / Q000648:ultrastructure; D009474:Neurons / Q000648:ultrastructure*; D013661:Tay-Sachs Disease / Q000175:diagnosis / Q000473:pathology*