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The Leloir pathway is a metabolic pathway for the catabolism of D-galactose. It is named after Luis Federico Leloir, who first described it.

In the first step, galactose mutarotase facilitates the conversion of β-D-galactose to α-D-galactose since this is the active form in the pathway. Next, α-D-galactose is phosphorylated by galactokinase to galactose 1-phosphate. In the third step, D-galactose-1-phosphate uridylyltransferase converts galactose 1-phosphate to UDP-galactose using UDP-glucose as the uridine diphosphate source. Finally, UDP-galactose 4-epimerase recycles the UDP-galactose to UDP-glucose for the transferase reaction. Additionally, phosphoglucomutase converts the D-glucose 1-phosphate to D-glucose 6-phosphate.

See also

• Anomer
• Mutarotation
• Epimer
  • Epimerase

References

1. Trucco, R.E.; Caputto, R; Leloir, L.F.; Mittelman, N (1948). "Galactokinase". Arch. Biochem. 18 (1): 137–146. PMID 18871223.
2. Caputto, R; Leloir, L.F.; Trucco, R.E.; Cardini, C.E.; Paladini, A C (1949). "The enzymatic transformation of galactose into glucose derivatives". J. Biol. Chem. 179 (1): 497–498. doi:10.1016/S0021-9258(18)56863-0. hdl:11336/135761. PMID 18119268.
3. Cardini, C. E.; Paladini, A. C.; Caputto, R.; Leloir, L. F. (1950). "Uridine Diphosphate Glucose: The Coenzyme of the Galactose–Glucose Phosphate Isomerization". Nature. 165 (4188): 191–192. Bibcode:1950Natur.165..191C. doi:10.1038/165191a0. hdl:11336/140707. S2CID 44975916.
4. Leloir, Luis F. (1951). "The enzymatic transformation of uridine diphosphate glucose into a galactose derivative". Archives of Biochemistry and Biophysics. 33 (2): 186–190. doi:10.1016/0003-9861(51)90096-3. hdl:11336/140700. PMID 14885999.
5. Holden HM, Rayment I, Thoden JB (November 2003). "Structure and function of enzymes of the Leloir pathway for galactose metabolism". J. Biol. Chem. 278 (45): 43885–8. doi:10.1074/jbc.R300025200. PMID 12923184.
6. Frey PA (March 1996). "The Leloir pathway: a mechanistic imperative for three enzymes to change the stereochemical configuration of a single carbon in galactose". FASEB J. 10 (4): 461–70. doi:10.1096/fasebj.10.4.8647345. PMID 8647345. S2CID 13857006. Retrieved 2011-04-01.

• Metabolic pathways
• Inborn errors of metabolism
• Galactose