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  • Title: Free amino acids in liver of patients with homocystinuria due to cystathionine synthase deficiency: effects of vitamin B6.
    Author: Rassin DK, Longhi RC, Gaull GE.
    Journal: J Pediatr; 1977 Oct; 91(4):574-7. PubMed ID: 908976.
    Abstract:
    Patients with homocystinuria due to cystathionine synthase deficiency do not have free homocystine in the liver when it is present in high concentrations in the plasma and the urine. The liver of these patients is capable of maintaining normal concentrations of cystine at a time when the plasma cystine concentration is severely reduced. There is an increase in the methionine concentration of the liver which is reduced to normal concentrations during pyridoxine therapy.
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