Cystathioninuria: nature of the defect.

Abstract	A homogenate of liver obtained by biopsy from two patients with cystathioninuria, an inborn error of metabolism, cleaved radioactive cystathionine only slightly until an excess of pyridoxal phosphate was added. The apoenzyme failed to bind the coenzyme normally. Pyridoxine therapy of familial cystathioninuria thus has a sound basis.
Authors	G W Frimpter
Journal	Science (New York, N.Y.) (Science) Vol. 149 Issue 3688 Pg. 1095-6 (Sep 03 1965) ISSN: 0036-8075 [Print] United States
PMID	5826521 (Publication Type: Journal Article)
Chemical References	Tritium Cystine Pyridoxal Phosphate Hydro-Lyases
Topics	Amino Acid Metabolism, Inborn Errors Child Child, Preschool Cystine Humans Hydro-Lyases In Vitro Techniques Liver (metabolism) Pyridoxal Phosphate Radiometry Subcellular Fractions Tritium Urine

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