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Agarose gel isoelectrofocusing of UDP-galactose pyrophosphorylase and galactose-1-phosphate uridyltransferase. Developmental aspect of UDP-galactose pyrophosphorylase.
Clin Chim Acta. 1987 Jun 30; 166(1):27-35.CC

Abstract

The uridine diphosphogalactose pyrophosphorylase activity has been determined in human adult and fetal tissues as well as blood of various ages by measurement of UDP-galactose production from gal-1-p and UTP. The highest activity was found from adult liver in which the specific activity was about 5% of the gal-1-p uridyltransferase activity. In general adult tissues had a somewhat higher activity than the corresponding fetal tissues except erythrocytes in which fetuses had a 5-10 times higher activity than adults. From normal blood the pyrophosphorylase activity in erythrocytes decreased with age, but in the case of galactosemia the decrease with age was not distinct. According to agarose gel isoelectrofocusing studies, at least two isozyme forms for UDP-galactose pyrophosphorylase exist with the activity bands between pH 6.0-6.15. The patterns of AGIF bands of pyrophosphorylase varied according to the age of the samples, suggesting the development of the isozyme forms of pyrophosphorylase to be age-dependent. Uridyltransferase, on the other hand, resolved into multiple bands between pH 5.1-5.6 on agarose gels and the patterns varied according to the variants but not to the age. Significance of the decrease in the pyrophosphorylase activity in erythrocytes with age as well as of the difference in AGIF bands between normal and the galactosemic were discussed with regard to the pathology of classical galactosemia.

Authors

Shin YS
No affiliation info available
Niedermeier HP
No affiliation info available
Endres W
No affiliation info available
Schaub J
No affiliation info available
Weidinger S
No affiliation info available

MeSH

AdolescentAdultAge FactorsFetusGalactosemiasHumansInfantInfant, NewbornIsoelectric FocusingLiverMiddle AgedNucleotidyltransferasesUTP-Hexose-1-Phosphate Uridylyltransferase

Pub Type(s)

Journal Article

Language

eng

PubMed ID

3038381

Citation

Shin, Y S., et al. "Agarose Gel Isoelectrofocusing of UDP-galactose Pyrophosphorylase and Galactose-1-phosphate Uridyltransferase. Developmental Aspect of UDP-galactose Pyrophosphorylase." Clinica Chimica Acta; International Journal of Clinical Chemistry, vol. 166, no. 1, 1987, pp. 27-35.
Shin YS, Niedermeier HP, Endres W, et al. Agarose gel isoelectrofocusing of UDP-galactose pyrophosphorylase and galactose-1-phosphate uridyltransferase. Developmental aspect of UDP-galactose pyrophosphorylase. Clin Chim Acta. 1987;166(1):27-35.
Shin, Y. S., Niedermeier, H. P., Endres, W., Schaub, J., & Weidinger, S. (1987). Agarose gel isoelectrofocusing of UDP-galactose pyrophosphorylase and galactose-1-phosphate uridyltransferase. Developmental aspect of UDP-galactose pyrophosphorylase. Clinica Chimica Acta; International Journal of Clinical Chemistry, 166(1), 27-35.
Shin YS, et al. Agarose Gel Isoelectrofocusing of UDP-galactose Pyrophosphorylase and Galactose-1-phosphate Uridyltransferase. Developmental Aspect of UDP-galactose Pyrophosphorylase. Clin Chim Acta. 1987 Jun 30;166(1):27-35. PubMed PMID: 3038381.
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TY - JOUR T1 - Agarose gel isoelectrofocusing of UDP-galactose pyrophosphorylase and galactose-1-phosphate uridyltransferase. Developmental aspect of UDP-galactose pyrophosphorylase. AU - Shin,Y S, AU - Niedermeier,H P, AU - Endres,W, AU - Schaub,J, AU - Weidinger,S, PY - 1987/6/30/pubmed PY - 1987/6/30/medline PY - 1987/6/30/entrez SP - 27 EP - 35 JF - Clinica chimica acta; international journal of clinical chemistry JO - Clin Chim Acta VL - 166 IS - 1 N2 - The uridine diphosphogalactose pyrophosphorylase activity has been determined in human adult and fetal tissues as well as blood of various ages by measurement of UDP-galactose production from gal-1-p and UTP. The highest activity was found from adult liver in which the specific activity was about 5% of the gal-1-p uridyltransferase activity. In general adult tissues had a somewhat higher activity than the corresponding fetal tissues except erythrocytes in which fetuses had a 5-10 times higher activity than adults. From normal blood the pyrophosphorylase activity in erythrocytes decreased with age, but in the case of galactosemia the decrease with age was not distinct. According to agarose gel isoelectrofocusing studies, at least two isozyme forms for UDP-galactose pyrophosphorylase exist with the activity bands between pH 6.0-6.15. The patterns of AGIF bands of pyrophosphorylase varied according to the age of the samples, suggesting the development of the isozyme forms of pyrophosphorylase to be age-dependent. Uridyltransferase, on the other hand, resolved into multiple bands between pH 5.1-5.6 on agarose gels and the patterns varied according to the variants but not to the age. Significance of the decrease in the pyrophosphorylase activity in erythrocytes with age as well as of the difference in AGIF bands between normal and the galactosemic were discussed with regard to the pathology of classical galactosemia. SN - 0009-8981 UR - https://www.unboundmedicine.com/medline/citation/3038381/Agarose_gel_isoelectrofocusing_of_UDP_galactose_pyrophosphorylase_and_galactose_1_phosphate_uridyltransferase__Developmental_aspect_of_UDP_galactose_pyrophosphorylase_ DB - PRIME DP - Unbound Medicine ER -