Homocysteine and cysteine loads in patients with homocystinuria due to cystathionine synthase deficiency: effects of vitamin B-6
| Autor: | John A. Sturman, Gerald E. Gaull, Hans J. Sternowsky, Riccardo C. Longhi, David K. Rassin |
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| Rok vydání: | 1977 |
| Předmět: |
Male
medicine.medical_specialty Time Factors Homocysteine Clinical Biochemistry Cystine Cystathionine beta-Synthase Homocystinuria Biochemistry chemistry.chemical_compound Internal medicine medicine Humans Cysteine Disulfides Cysteine metabolism Hydro-Lyases Methionine Cystinuria ATP synthase biology Homocystine business.industry Biochemistry (medical) Pyridoxine General Medicine medicine.disease Endocrinology chemistry biology.protein Female business |
| Zdroj: | Clinica chimica acta; international journal of clinical chemistry. 79(1) |
| ISSN: | 0009-8981 |
| Popis: | The metabolic response of patients with homocystinuria due to cystabhionine 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. |
| Databáze: | OpenAIRE |
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