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l pipecolic acid/atrofija

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L-alpha-aminoadipate inhibits kynurenate synthesis in rat brain hippocampus and tissue culture.

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Intracerebral administration of L-alpha-aminoadipic acid (L-AAA) at 500 mg/kg body weight to rats caused a complex behavioral change with sporadic wet-dog shakes. Animals developed severe limbic seizures between 1 and 6 h after L-AAA injection, characterized by generalized convulsions. Twenty days

A new peroxisomal disease with impaired phytanic and pipecolic acid oxidation.

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Phytanic acid (PA) accumulates in patients with adult Refsum disease (ARD) and with peroxisomal disorders. In three related patients with ARD, PA levels were moderately increased in plasma, whereas phytanic oxidation was severely deficient in the fibroblasts. Two of these patients had a significant
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