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A new variant of galactosemia: galactose-1-phosphate uridylytransferase sensitive to product inhibition by glucose 1-phosphate.
Pediatr Res. 1980 May; 14(5):729-34.PR

Abstract

In the erythrocytes of a patient with the clinical symptoms of galactosemia, a galactose-1-phosphate uridylyltransferase with abnormal kinetics was observed. Under standard assay conditions, the uridylyltransferase activity was almost normal initially and became completely inactivated within 30 min. The abnormal kinetics could be ascribed to a product inhibition by glucose 1-phosphate. The inhibition was produced by a variety of sugar phosphates, the most potent of which proved to be glucose 1-phosphate, mannose 1-phosphate, and fructose 6-phosphate. The variant galactose-1-phosphate uridylyltransferase was further characterized by a lowered affinity towards galactose 1-phosphate, non-Michaelis-Menten kinetics towards UDP-glucose, an increased thermal stability, and complete inactivity upon Cellogel electrophoresis.

Authors

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Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

6247691

Citation

Lang, A, et al. "A New Variant of Galactosemia: Galactose-1-phosphate Uridylytransferase Sensitive to Product Inhibition By Glucose 1-phosphate." Pediatric Research, vol. 14, no. 5, 1980, pp. 729-34.
Lang A, Groebe H, Hellkuhl B, et al. A new variant of galactosemia: galactose-1-phosphate uridylytransferase sensitive to product inhibition by glucose 1-phosphate. Pediatr Res. 1980;14(5):729-34.
Lang, A., Groebe, H., Hellkuhl, B., & von Figura, K. (1980). A new variant of galactosemia: galactose-1-phosphate uridylytransferase sensitive to product inhibition by glucose 1-phosphate. Pediatric Research, 14(5), 729-34.
Lang A, et al. A New Variant of Galactosemia: Galactose-1-phosphate Uridylytransferase Sensitive to Product Inhibition By Glucose 1-phosphate. Pediatr Res. 1980;14(5):729-34. PubMed PMID: 6247691.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A new variant of galactosemia: galactose-1-phosphate uridylytransferase sensitive to product inhibition by glucose 1-phosphate. AU - Lang,A, AU - Groebe,H, AU - Hellkuhl,B, AU - von Figura,K, PY - 1980/5/1/pubmed PY - 1980/5/1/medline PY - 1980/5/1/entrez SP - 729 EP - 34 JF - Pediatric research JO - Pediatr Res VL - 14 IS - 5 N2 - In the erythrocytes of a patient with the clinical symptoms of galactosemia, a galactose-1-phosphate uridylyltransferase with abnormal kinetics was observed. Under standard assay conditions, the uridylyltransferase activity was almost normal initially and became completely inactivated within 30 min. The abnormal kinetics could be ascribed to a product inhibition by glucose 1-phosphate. The inhibition was produced by a variety of sugar phosphates, the most potent of which proved to be glucose 1-phosphate, mannose 1-phosphate, and fructose 6-phosphate. The variant galactose-1-phosphate uridylyltransferase was further characterized by a lowered affinity towards galactose 1-phosphate, non-Michaelis-Menten kinetics towards UDP-glucose, an increased thermal stability, and complete inactivity upon Cellogel electrophoresis. SN - 0031-3998 UR - https://www.unboundmedicine.com/medline/citation/6247691/A_new_variant_of_galactosemia:_galactose_1_phosphate_uridylytransferase_sensitive_to_product_inhibition_by_glucose_1_phosphate_ L2 - https://doi.org/10.1203/00006450-198005000-00005 DB - PRIME DP - Unbound Medicine ER -