Defects in Galactose Metabolism and Glycoconjugate Biosynthesis in a UDP-Glucose Pyrophosphorylase-Deficient Cell Line Are Reversed by Adding Galactose to the Growth Medium

Defects in Galactose Metabolism and Glycoconjugate Biosynthesis in a UDP-Glucose Pyrophosphorylase-Deficient Cell Line Are Reversed by Adding Galactose to the Growth Medium

UDP-glucose (UDP-Glc) is synthesized by <i>UGP2</i>-encoded UDP-Glc pyrophosphorylase (UGP) and is required for glycoconjugate biosynthesis and galactose metabolism because it is a uridyl donor for galactose-1-P (Gal1P) uridyltransferase. Chinese hamster lung fibroblasts harboring a hypo...

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Bibliographic Details
Main Authors: Christelle Durrant, Jana I. Fuehring, Alexandra Willemetz, Dominique Chrétien, Giusy Sala, Riccardo Ghidoni, Abram Katz, Agnès Rötig, Monica Thelestam, Myriam Ermonval, Stuart E. H. Moore
Format: Article
Language:English
Published: MDPI AG 2020-03-01
Series:International Journal of Molecular Sciences
Subjects:
udp-glucose
udp-galactose
ugp2
protein glycosylation
prion protein
glycosphingolipid
galactose
congenital disorders of glycosylation (cdgs)
developmental epileptic encephalopathies (dees)
Online Access:https://www.mdpi.com/1422-0067/21/6/2028

Internet

https://www.mdpi.com/1422-0067/21/6/2028

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