Homocysteine and cysteine loads in patients with homocystinuria due to cystathionine synthase deficiency: Effects of vitamin B-6

David K. Rassin; Riccardo C. Longhi; Hans J. Sternowsky; John A. Sturman; Gerald E. Gaull

doi:10.1016/0009-8981(77)90478-8

Homocysteine and cysteine loads in patients with homocystinuria due to cystathionine synthase deficiency: Effects of vitamin B-6

David K. Rassin, Riccardo C. Longhi, Hans J. Sternowsky, John A. Sturman, Gerald E. Gaull

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

Abstract

The metabolic response of patients with homocystinuria due to cystathionine synthase deficiency to oral loads of homocysteine indicates: that even severely affected patients with homocystinuria have pools of cystine in their tissues; that control of sulfur amino acid metabolism favors increased concentrations of methionine rather than homocystine in the plasma; and that even patients who apparently are not B-6-responsive respond differently to the loads of homocysteine when challenged during B-6-treatment compared with their response before B-6 treatment. Loading tests with homocysteine indicate that B-6 treatment be of some benefit even in individuals who do not have an obvious biochemical response to such therapy.

Original language	English (US)
Pages (from-to)	197-210
Number of pages	14
Journal	Clinica Chimica Acta
Volume	79
Issue number	1
DOIs	https://doi.org/10.1016/0009-8981(77)90478-8
State	Published - Aug 15 1977
Externally published	Yes

ASJC Scopus subject areas

Biochemistry
Clinical Biochemistry
Biochemistry, medical

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abstract = "The metabolic response of patients with homocystinuria due to cystathionine synthase deficiency to oral loads of homocysteine indicates: that even severely affected patients with homocystinuria have pools of cystine in their tissues; that control of sulfur amino acid metabolism favors increased concentrations of methionine rather than homocystine in the plasma; and that even patients who apparently are not B-6-responsive respond differently to the loads of homocysteine when challenged during B-6-treatment compared with their response before B-6 treatment. Loading tests with homocysteine indicate that B-6 treatment be of some benefit even in individuals who do not have an obvious biochemical response to such therapy.",

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AU - Longhi, Riccardo C.

AU - Sternowsky, Hans J.

AU - Sturman, John A.

AU - Gaull, Gerald E.

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AB - The metabolic response of patients with homocystinuria due to cystathionine synthase deficiency to oral loads of homocysteine indicates: that even severely affected patients with homocystinuria have pools of cystine in their tissues; that control of sulfur amino acid metabolism favors increased concentrations of methionine rather than homocystine in the plasma; and that even patients who apparently are not B-6-responsive respond differently to the loads of homocysteine when challenged during B-6-treatment compared with their response before B-6 treatment. Loading tests with homocysteine indicate that B-6 treatment be of some benefit even in individuals who do not have an obvious biochemical response to such therapy.

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Homocysteine and cysteine loads in patients with homocystinuria due to cystathionine synthase deficiency: Effects of vitamin B-6

Abstract

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