Hallervorden-Spatz disease: Cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus

We describe neurochemical abnormalities found in the brains of 2 patients with autopsy‐confirmed Hallervorden‐Spatz (HS) disease. In 1 patient, contents of cystine and of glutathione‐cysteine mixed disulfide in the globus pallidus were markedly elevated above values for appropriate control subjects....

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Veröffentlicht in:	Annals of neurology 1985-10, Vol.18 (4), p.482-489
Hauptverfasser:	Perry, Thomas L, Norman, Margaret G., Yong, Voon Wee, Whiting, Sharon, Crichton, John U., Hansen, Shirley, Kish, Stephen J.
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Amino Acids - cerebrospinal fluid Amino Acids - metabolism Basal Ganglia Diseases - metabolism Biological and medical sciences Brain Chemistry Cerebral Cortex - enzymology Cerebral Cortex - metabolism Child Choline O-Acetyltransferase - metabolism Cysteine - metabolism Cysteine Dioxygenase Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Dioxygenases Dopamine - metabolism Female Globus Pallidus - enzymology Globus Pallidus - metabolism Globus Pallidus - pathology Humans Male Medical sciences Middle Aged Neurology Oxygenases - deficiency Oxygenases - metabolism Pantothenate Kinase-Associated Neurodegeneration - enzymology Pantothenate Kinase-Associated Neurodegeneration - metabolism Pantothenate Kinase-Associated Neurodegeneration - pathology Tissue Distribution
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