The stereochemistry for hydrogen removal from pyridoxamine 5'-phosphate with liver pyridoxine (pyridoxamine)-5'-phosphate oxidase was examined to determine whether or not there are significant steric constraints at the substrate region of the active site of the oxidase. For this, pyridoxal 5'-phosphate was reduced with tritium-labeled sodium borohydride in ammoniacal solution to yield racemical...

A full-term female neonate developed focal motor seizures at 1 hour of life, followed by paroxysmal nonepileptic abnormal eye–head movements on day 5 (video 1). CSF revealed mild hypoglycorrhachia, borderline low CSF:serum glucose, and pyridoxal-5′-phosphate (P5P). Genetic testing showed compound heterozygous mutations in the pyridoxamine 5′-phosphate oxidase ( PNPO ) gene consistent with P5P-d...

Although pyridoxal phosphate is known to inhibit gelation of purified hemoglobin S, antisickling activity has never been demonstrated for intact erythrocytes. We incubated washed erythrocytes at 37 degrees C either in buffer alone, or with added pyridoxal phosphate or pyridoxal, washed these cells, suspended them in untreated buffer, and compared the percent modified hemoglobin, the oxygen affi...

The catalytic activity concentrations of aspartate aminotransferase (EC 2.6.1.1) and alanine aminotransferase (EC 2.6.1.2) in several commercial control sera were determined at three different temperatures. Although these sera were of various biological origins, the temperature-conversion factors calculated are nearly identical to those for native human sera. The proportional increase of the ac...

In studies with pyridoxine and other B(6) compounds in blood, the active forms pyridoxal and pyridoxal phosphate were measured by differential assays using Lactobacillus casei. Red cell uptake of tritiated pyridoxine was also measured. A new metabolic pathway for conversion of pyridoxine to active forms was demonstrated in red cells. In vivo studies in normal subjects suggested that pyridoxine ...