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On the enzymic defects in hereditary tyrosinemia.
TLDR
It is suggested that the severe liver and kidney damage in hereditary tyrosinemia may be due to the accumulation of these tyrosine metabolites and that the primary enzyme defect in hereditary Tyrosinema may be decreased activity of fumarylacetoacetase.
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A method for the determination of carnitine in the picomole range.
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The turnover of cholic acid in man: bile acids and steroids.
TLDR
From values of the quantitative composition of the bile the chenodeoxycholics and deoxycholic acid pools have been calculated and the specific activity of the circulating cholic acid has been followed.
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The Formation and Degradation of Carnitine in Pseudomonas
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Metabolic interconversions of different forms of vitamin B6.
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The mechanism of enzymic formation of homogentisate from p-hydroxyphenylpyruvate.
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Characterization of 4-hydroxyphenylpyruvate dioxygenase. Primary structure of the Pseudomonas enzyme.
TLDR
The primary structure of Pseudomonas 4-hydroxyphenylpyruvate dioxygenase was determined and secondary structure predictions suggest an alpha/beta mixed structure, fairly typical of globular proteins, without long segments of hydrophobicity or charge.
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Purification and some properties of human 4-hydroxyphenylpyruvate dioxygenase (I).
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Gamma-butyrobetaine hydroxylase from Pseudomonas sp AK 1.
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3-Methylglutaconic aciduria in two infants.
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