Beutler, E., and Baluda, M. C. (1966). Improved method for measuring galactose-l-phosphate uridyl transferase activity of erythrocytes. Clinica Chimica Acta, 13, 369-379. Federhol, M. K. (1969). Quantitative studies on the inherited variants of serum alpha-l-antitrypsin. Scandinavian Journal of Clinical and Laboratory Investigation, 23, 97-103. Lejeune, J., Berger, R., Lafourcase, J., and Retho...

Beutler, E., and Baluda, M. C. (1966). Improved method for measuring galactose-l-phosphate uridyl transferase activity of erythrocytes. Clinica Chimica Acta, 13, 369-379. Federhol, M. K. (1969). Quantitative studies on the inherited variants of serum alpha-l-antitrypsin. Scandinavian Journal of Clinical and Laboratory Investigation, 23, 97-103. Lejeune, J., Berger, R., Lafourcase, J., and Retho...

Galactosemia is an autosomal recessive disorder, which caused by a deficiency of one of three enzymes that involved in the metabolism of galactose; galactokinase (GALK), galactose-1-phosphate uridyltransferase (GALT) and uridine-diphosphate galactose-4’epimerase (GALE). Clinical manifestations including poor feeding, jaundice, hepatomegaly, vomiting, hypoglycemia, convulsions, failure to thrive...

Suspension cultures of neoplastic mouse mast cells were used to obtain large quantities of a homogeneous cell population as starting material for cell fractionation. A Golgi fraction was prepared by slight modification of established techniques and identified by electron microscopy. Assay of galactosyl transferase activity using ovalbumin, desialylated degalactosylated orosomucoid, and N-acetyl...

Glycoproteins containing N-linked oligosaccharides were prepared from plasma and liver microsomes of rats aged 0-5 weeks, and galactose and sialic acid content were determined. The sialic acid/galactose ratios in plasma membrane N-glycans remained at about 1 throughout the postnatal period, suggesting that most of the galactose residues are sialylated. In the same way, it was suggested that mos...

RNAi is a conserved mechanism in which small RNAs induce silencing of complementary targets. How Argonaute-bound small RNAs are targeted for degradation is not well understood. We show that the adenyl-transferase Cid14, a member of the TRAMP complex, and the uridyl-transferase Cid16 add non-templated nucleotides to Argonaute-bound small RNAs in fission yeast. The tailing of Argonaute-bound smal...

The biological interconversion of galactose and glucose takes place only by way of the Leloir pathway and requires the three enzymes galactokinase, galactose-1-P uridylyltransferase, and UDP-galactose 4-epimerase. The only biological importance of these enzymes appears to be to provide for the interconversion of galactosyl and glucosyl groups. Galactose mutarotase also participates by producing...