Ultrastructural changes in rat optic nerve associated with hyperphenylalaninemia induced by para-chlorophenylalanine and phenylalanine.
The morphologic effects of hyperphenylalaninemia induced by treatment with para-chlorophenylalanine (PCP) plus phenylalanine on optic nerve were studied in developing F344 rats. PCP and phenylalanine were infected daily between days 5 and 20 days of life. At 20 days optic nerve of treated animals, as compared with saline-injected controls, showed enhanced neuroglial activity with broad astrocytic septae and debris-laden oligodendrocytes. In specimens obtained long after treatment with PCP and phenylalanine, continuing gliosis with evidence of focally abnormal myelination and axonal degeneration were observed. The results are consistent with a metabolic insult sustained in early development by astrocytes and oligodendrocytes, and are considered in relation to other work in experimental hyperphenylalaninemia and to human phenylketonuria.
D000818:Animals; D001253:Astrocytes / Q000648:ultrastructure; D001369:Axons / Q000648:ultrastructure; D003593:Cytoplasm / Q000648:ultrastructure; D004195:Disease Models, Animal; D004721:Endoplasmic Reticulum / Q000648:ultrastructure; D010134:Fenclonine; D006056:Golgi Apparatus / Q000648:ultrastructure; D006801:Humans; D008854:Microscopy, Electron; D009186:Myelin Sheath / Q000648:ultrastructure; D009410:Nerve Degeneration / Q000187:drug effects; D009836:Oligodendroglia / Q000648:ultrastructure; D009900:Optic Nerve / Q000648:ultrastructure*; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000139:chemically induced / Q000473:pathology*; D051381:Rats; D011916:Rats, Inbred F344; D012965:Sodium Chloride
D012965:Sodium Chloride; D010649:Phenylalanine; D010134:Fenclonine