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 journalArticle

5 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)197-210
Number of pages14
JournalClinica Chimica Acta
Volume79
Issue number1
DOIs
StatePublished - Aug 15 1977
Externally publishedYes

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Vitamin B 6 Deficiency
Cystathionine
Homocystinuria
Vitamin B 6
Homocysteine
Cysteine
Homocystine
Sulfur Amino Acids
Cystine
Metabolism
Methionine
Therapeutics
Tissue
Plasmas

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry

Cite this

Homocysteine and cysteine loads in patients with homocystinuria due to cystathionine synthase deficiency : Effects of vitamin B-6. / Rassin, David K.; Longhi, Riccardo C.; Sternowsky, Hans J.; Sturman, John A.; Gaull, Gerald E.

In: Clinica Chimica Acta, Vol. 79, No. 1, 15.08.1977, p. 197-210.

Research output: Contribution to journalArticle

Rassin, David K. ; Longhi, Riccardo C. ; Sternowsky, Hans J. ; Sturman, John A. ; Gaull, Gerald E. / Homocysteine and cysteine loads in patients with homocystinuria due to cystathionine synthase deficiency : Effects of vitamin B-6. In: Clinica Chimica Acta. 1977 ; Vol. 79, No. 1. pp. 197-210.
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