Pathochemical study of a case of hereditary cerebral degenerative disease--lipopigment accumulation and peroxidase deficiency in brain.
Ultrastructural study of autoptic cerebral tissue from a patient with hereditary degenerative disease of the nervous system revealed lipofuscin-like bodies and variable sized osmiophilic granular masses in neurons. Autofluorescent granules were also found in neurons. Biochemical study demonstrated a deficiency of diaminobenzidine peroxidase and the accumulation of lipopigment in cerebral tissue.
D001921:Brain / Q000201:enzymology*; D002648:Child; D005260:Female; D006801:Humans; D008062:Lipofuscin / Q000032:analysis; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism* / Q000473:pathology; D010544:Peroxidases / Q000172:deficiency*; D010860:Pigments, Biological / Q000032:analysis*
D008062:Lipofuscin; D010860:Pigments, Biological; D010544:Peroxidases; C037561:diaminobenzidine peroxidase