Cystathionase deficiency: the effect of cofactor on the stability of normal and abnormal enzyme from lymphoid cell lines.

Heating at 70 degrees C with and without added pyridoxal phosphate (PLP) had strikingly different effects on cystathionase protein from normal long-term lymphoid cell lines and on the enzymes from cells derived from patients with vitamin-B6-responsive cystathioninuria. PLP added to extracts of normal cells afforded complete protection against heat inactivation, whereas inactivation of the cystathionase protein in extracts obtained from two cystathioninuric lines was greater in the presence of PLP than in its absence.