Superoxide dismutase isoenzymes in cerebrospinal fluid and plasma from patients with neuronal ceroid-lipofuscinoses.
The neuronal ceroid-lipofuscinoses is a group of diseases characterized by a widespread accumulation in the body of pigments believed to be end-products of lipid-peroxidation damaged organelles. It was recently shown that cerebrospinal fluid from patients with infantile and juvenile neuronal ceroid-lipofuscinosis were less protective against superoxide radical-induced hydroxyl radical formation compared with controls. The content of superoxide dismutase isoenzymes in cerebrospinal fluid and in plasma from patients with different forms of neuronal ceroid-lipofuscinosis was analysed. No significant difference from controls could be demonstrated in samples from patients with juvenile neuronal ceroid-lipofuscinosis. The few samples from patients with infantile and late infantile neuronal ceroid-lipofuscinosis analysed all fell within the range defined by the controls.
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Marklund
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Heiskala
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T
Westermarck
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P
Santavuori
D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007527:Isoenzymes / Q000097:blood / Q000134:cerebrospinal fluid*; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000201:enzymology*; D013482:Superoxide Dismutase / Q000097:blood / Q000134:cerebrospinal fluid*
D007527:Isoenzymes; D013482:Superoxide Dismutase