Skip to main content

UDP-Glucose-hexose-1-phosphate uridylyltransferase

  • Chapter
Springer Handbook of Enzymes

Part of the book series: Springer Handbook of Enzymes ((HDBKENZYMES,volume 38))

  • 973 Accesses

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 259.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 329.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 329.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Saito, S.; Ozutsumi, M.; Kurahashi, K.: Galactose 1-phosphate uridylyltransferase of Escherichia coli. II. Further purification and characterization. J. Biol. Chem., 242, 2362–2368 (1967)

    PubMed  CAS  Google Scholar 

  2. Arabshahi, A.; Brody, R.S.; Smallwood, A.; Tsai, T.-C; Frey, P.A.: Galactose-1-phosphate uridylyltransferase. Purification of the enzyme and stereochemical course of each step of the double-displacement mechanism. Biochemistry, 25, 5583–5589 (1986)

    Article  PubMed  CAS  Google Scholar 

  3. Wedekind, J.E.; Frey, P.A.; Rayment, I.: Crystallization and preliminary crystallographic analysis of galactose-1-phosphate uridylyltransferase from Escherichia coli. Acta Crystallogr. Sect. D, D50, 329–331 (1994)

    Article  CAS  Google Scholar 

  4. Lee, L.; Kimura, A.; Tochikura, T.: Purification and properties of hexose 1-phosphate uridylyltransferase from Bifidobacterium bifidum. Agric. Biol. Chem., 42, 723–730 (1978)

    CAS  Google Scholar 

  5. Segawa, T.; Fukasawa, T.: The enzymes of the galactose cluster in Saccharomyces cerevisiae. Purification and characterization of galactose-1-phosphate uridylyltransferase. J. Biol. Chem., 254, 10707–10709 (1979)

    PubMed  CAS  Google Scholar 

  6. Mayes, J.S.; Hansen, R.G.: Galactose 1-phosphate uridyl transferase. Methods Enzymol., 9, 708–713 (1966)

    CAS  Google Scholar 

  7. Mayes, J.S.: Purification, properties, and isozyme pattern of galactose-1-phosphate uridyl transferase from calf liver. Arch. Biochem. Biophys., 172, 715–720 (1976)

    Article  PubMed  CAS  Google Scholar 

  8. Helmer, G.R.; Williams, V.P.: Purification of galactose-1-phosphate uridylyltransferase from human placenta. Arch. Biochem. Biophys., 210, 573–580 (1981)

    Article  PubMed  CAS  Google Scholar 

  9. Williams, V.P.; Helmer, G.R.; Fried, C: Human galactose-1-phosphate uridylyltransferase: purification and comparison of the red blood cell and placental enzymes. Arch. Biochem. Biophys., 216, 503–511 (1982)

    Article  PubMed  CAS  Google Scholar 

  10. Williams, V.P.: Purification and some properties of galactose 1-phosphate uridylyltransferase from human red cells. Arch. Biochem. Biophys., 191, 182–191 (1978)

    Article  PubMed  CAS  Google Scholar 

  11. Kelly, S.; Sweeney, P.; Schedlbauer, L.: Galactose-1-phosphate uridyl transferase activity in red cells of various animal species. Experientia, 37, 550–553 (1981)

    Article  PubMed  CAS  Google Scholar 

  12. Chowdhury, R.R.: Purification & some properties of galactose-1-phosphate uridylyl transferase from E. coli. Indian J. Biochem. Biophys., 16, 273–277 (1979)

    PubMed  CAS  Google Scholar 

  13. Ellis, G.; Goldberg, D.M.: Galactose-1-phosphate uridylytransferase. UDP-glucose:α-D-galactose-1-phosphate uridylyltransferase, EC 2.7.7.12. Methods Enzym. Anal., 3rd. Ed. (Bergmeyer, H.U., ed.), 3, 560–571 (1983)

    Google Scholar 

  14. Banroques, J.; Gregori, C; Schapira, F.: Purification and characterization of human erythrocyte uridylyl transferase. Biochim. Biophys. Acta, 657, 374–382 (1981)

    PubMed  CAS  Google Scholar 

  15. Fukasawa, T.; Segawa, T.; Nogi, Y.: Uridine diphosphate glucose-4-epimerase and galactose-1-phosphate uridylyltransferase from Saccharomyces cerevisiae. Methods Enzymol., 89, 584–592 (1982)

    PubMed  CAS  Google Scholar 

  16. Frey, P.A.; Wong, L.-J.; Sheu, K.-F.; Yang, S.-L.: Galactose-1-phosphate uridylyltransferase: detection, isolation, and characterization of the uridylyl enzyme. Methods Enzymol., 87, 20–36 (1982)

    PubMed  CAS  Google Scholar 

  17. Arabshahi, A.; Ruzicka, F.J.; Geeganage, S.; Frey, P.A.: Standard free energies for uridylyl group transfer by hexose-1-P uridylyltransferase and UDP-hexose synthase and for the hydrolysis of uridine 5′-phosphoimidazolate. Biochemistry, 35, 3426–3428 (1996)

    Article  PubMed  CAS  Google Scholar 

  18. Daude, N.; Lestage, J.; Reichardt, J.K.; Petry, K.G.: Expression of galactose-1-phosphate uridyltransferase in the anterior pituitary of rat during the estrous cycle. Neuroendocrinology, 64, 42–48 (1996)

    PubMed  CAS  Google Scholar 

  19. Podskarbi, T.; Kohlmetz, T.; Gathof, B.S.; Kleinlein, B.; Bieger, W.P.; Gresser, U.; Shin, Y.S.: Molecular characterization of Duarte-1 and Duarte-2 variants of galactose-1-phosphate uridyltransferase. J. Inher. Metab. Dis., 19, 638–644 (1996)

    Article  PubMed  CAS  Google Scholar 

  20. Wells, L.; Fridovich-Keil, J.L.: Biochemical characterization of the S135L allele of galactose-1-phosphate uridylyltransferase associated with galactosaemia. J. Inher. Metab. Dis., 20, 633–642 (1997)

    Article  PubMed  CAS  Google Scholar 

  21. Ruzicka, F.J.; Geeganage, S.; Frey, P.A.: Kinetic mechanism of UDP-hexose synthase, a point variant of hexose-1-phosphate uridylyltransferase from Escherichia coli. Biochemistry, 37, 11385–11392 (1998)

    Article  PubMed  CAS  Google Scholar 

  22. Lai, K.; Langley, S.D.; Dembure, P.P.; Hjelm, L.N.; Elsas, L.J., 2nd: Duarte allele impairs biostability of galactose-1-phosphate uridyltransferase in human lymphoblasts. Hum. Mutat., 11, 28–38 (1998)

    Article  PubMed  CAS  Google Scholar 

  23. Lai, K.; Willis, A.C.; Elsas, L.J.: The biochemical role of glutamine 188 in human galactose-1-phosphate uridyltransferase. J. Biol. Chem., 274, 6559–6566 (1999)

    Article  PubMed  CAS  Google Scholar 

  24. Feusi, M.E.S.; Burton, J.D.; Williamson, J.D.; Pharr, D.M.: Galactosyl-sucrose metabolism and UDP-galactose pyrophosphorylase from Cucumis melo L. fruit. Physiol. Plant., 106, 9–16 (1999)

    Article  CAS  Google Scholar 

  25. Leslie, N.D.; Bai, S.: Functional analysis of the mouse galactose-1-phosphate uridyl transferase (GALT)promoter. Mol. Genet. Metab., 72, 31–38 (2001)

    Article  PubMed  CAS  Google Scholar 

  26. Thoden, J.B.; Ruzicka, F.J.; Frey, P.A.; Rayment, I.; Holden, H.M.: Structural analysis of the H166G site-directed mutant of galactose 1-phosphate uridylyltransferase complexed with either UDP-glucose or UDP-galactose: detailed description of the nucleotide sugar binding site. Biochemistry, 36, 1212–1222 (1997)

    Article  PubMed  CAS  Google Scholar 

  27. Wedekind, J.E.; Frey, P.A.; Rayment, I.: The structure of nucleotidylated histidine-166 of galactose-1-phosphate uridylyltransferase provides insight into phosphoryl group transfer. Biochemistry, 35, 11560–11569 (1996)

    Article  PubMed  CAS  Google Scholar 

  28. Tamada, Y; Swanson, B.A.; Arabshahi, A.; Frey, P.A.: Preparation and characterization of a bifunctional fusion enzyme composed of UDP-galactose 4-epimerase and galactose-1-P uridylyltransferase. Bioconjugate Chem., 5, 660–665 (1994)

    Article  CAS  Google Scholar 

  29. Davit-Spraul, A.; Pourci, M.L.; Ng, K.H.; Soni, T.; Lemonnier, A.: Regulatory effects of galactose on galactose-1-phosphate uridyltransferase activity on human hepatoblastoma HepG2 cells. FEBS Lett., 354, 232–236 (1994)

    Article  PubMed  CAS  Google Scholar 

  30. Gross, W.; Schnarrenberger, C: Purification and characterization of a galactose-1-phosphate: UDP-glucose uridyltransferase from the red alga Galdieria sulphuraria. Eur. J. Biochem., 234, 258–263 (1995)

    Article  PubMed  CAS  Google Scholar 

  31. Ruzicka, F.J.; Wedekind, J.E.; Kim, J.; Rayment, I.; Frey, P.A.: Galactose-1-phosphate uridylyltransferase from Escherichia coli, a zinc and iron metalloenzyme. Biochemistry, 34, 5610–5617 (1995)

    Article  PubMed  CAS  Google Scholar 

  32. Shin, Y.S.; Zschocke, J.; Das, A.M.; Podskarbi, T.: Molecular and biochemical basis for variants and deficiency forms of galactose-1-phosphate uridyltransferase. J. Inher. Metab. Dis., 22, 327–329 (1999)

    Article  PubMed  CAS  Google Scholar 

  33. Quimby, B.B.; Wells, L.; Wilkinson, K.D.; Fridovich-Keil, J.L.: Functional requirements of the active site position 185 in the human enzyme galactose-1-phosphate uridylyltransferase. J. Biol. Chem., 271, 26835–26842 (1996)

    Article  PubMed  CAS  Google Scholar 

  34. Elsevier, J.P.; Wells, L.; Quimby, B.B.; Fridovich-Keil, J.L.: Heterodimer formation and activity in the human enzyme galactose-1-phosphate uridylyltransferase. Proc. Natl. Acad. Sci. USA, 93, 7166–7171 (1996)

    Article  PubMed  CAS  Google Scholar 

  35. Geeganage, S.; Frey, P.A.: Transient kinetics of formation and reaction of the uridylyl-enzyme form of galactose-1-P uridylyltransferase and its Q168R-variant: insight into the molecular basis of galactosemia. Biochemistry, 37, 14500–14507 (1998)

    Article  PubMed  CAS  Google Scholar 

  36. Geeganage, S.; Frey, P.A.: Significance of metal ions in galactose-1-phosphate uridylyltransferase: an essential structural zinc and a nonessential structural iron. Biochemistry, 38, 13398–13406 (1999)

    Article  PubMed  CAS  Google Scholar 

  37. Crews, C.; Wilkinson, K.D.; Wells, L.; Perkins, C.; Fridovich-Keil, J.L.: Functional consequence of substitutions at residue 171 in human galactose-1-phosphate uridylyltransferase. J. Biol. Chem., 275, 22847–22853 (2000)

    Article  PubMed  CAS  Google Scholar 

  38. Geeganage, S.; Ling, V.W.; Frey, P.A.: Roles of two conserved amino acid residues in the active site of galactose-1-phosphate uridylyltransferase: an essential serine and a nonessential cysteine. Biochemistry, 39, 5397–5404 (2000)

    Article  PubMed  CAS  Google Scholar 

  39. Riehman, K.; Crews, C; Fridovich-Keil, J.L.: Relationship between genotype, activity, and galactose sensitivity in yeast expressing patient alleles of human galactose-1-phosphate uridylyltransferase. J. Biol. Chem., 276, 10634–10640 (2001)

    Article  PubMed  CAS  Google Scholar 

  40. Christacos, N.C.; Fridovich-Keil, J.L.: Impact of patient mutations on heterodimer formation and function in human galactose-1-P uridylyltransferase. Mol. Genet. Metab., 76, 319–326 (2002)

    Article  PubMed  CAS  Google Scholar 

  41. Geeganage, S.; Frey, P.A.: Galactose-1-phosphate uridylyltransferase: kinetics of formation and reaction of uridylyl-enzyme intermediate in wild-type and specifically mutated uridylyltransferases. Methods Enzymol., 354, 134–148 (2002)

    Article  PubMed  CAS  Google Scholar 

Download references

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2007 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

(2007). UDP-Glucose-hexose-1-phosphate uridylyltransferase. In: Schomburg, D., Schomburg, I., Chang, A. (eds) Springer Handbook of Enzymes. Springer Handbook of Enzymes, vol 38. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-71526-9_17

Download citation

Publish with us

Policies and ethics