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Science 19 December 1975:
Vol. 190. no. 4220, pp. 1209 - 1211
DOI: 10.1126/science.1198108
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Articles

Science, Vol 190, Issue 4220, 1209-1211
Copyright © 1975 by American Association for the Advancement of Science

articles

Vitamin B6-responsive and -unresponsive cystathioninuria: two variant molecular forms

TA Pascal, GE Gaull, NG Beratis, BM Gillam, HH Tallan, and K Hirschhorn

Cystathionase activity in a lymphoid cell line extracts from a vitamin B6-responsive patient with cystathioninuria was increased strikingly by pyridoxal phosphate. Immunodiffusion with antiserum to human hepatic cystathionase showed identity between this cystathionase protein and cystathionase from an extract of normal lymphoid cells. Neither an increase in cystathionase activity nor immunochemical identity was found using extract of cells from a B6-unresponsive patient.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Diminished FAD Binding in the Y459H and V492E Antley-Bixler Syndrome Mutants of Human Cytochrome P450 Reductase.
C. C. Marohnic, S. P. Panda, P. Martasek, and B. S. Masters (2006)
J. Biol. Chem. 281, 35975-35982
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High-dose vitamin therapy stimulates variant enzymes with decreased coenzyme binding affinity (increased Km): relevance to genetic disease and polymorphisms.
B. N Ames, I. Elson-Schwab, and E. A Silver (2002)
Am. J. Clinical Nutrition 75, 616-658
   Abstract »    Full Text »    PDF »


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