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Leloir pathway

Metabolic pathway for the catabolism of D-galactose

The Leloir pathway is a metabolic pathway for the catabolism of D-galactose. It is named after Luis Federico Leloir, who first described it.{{cite journal | title = Galactokinase | last1 = Trucco | first1 = R.E. | last2 = Caputto | first2 = R | last3 = Leloir | first3 = L.F. | last4 = Mittelman | first4 = N | journal = Arch. Biochem. | volume = 18 | issue= 1 | pages= 137–146 | date = 1948 last1 = Caputto | first1 = R | last2 = Leloir | first2 = L.F. | last3 = Trucco | first3 = R.E. | last4 = Cardini | first4 =C.E. | last5 = Paladini | first5 =A C | journal = J. Biol. Chem. | volume= 179 | issue= 1 | pages= 497–498 | date = 1949| doi = 10.1016/S0021-9258(18)56863-0 | pmid = 18119268 | doi-access = free| hdl = 11336/135761| hdl-access = free}} | hdl-access=free

Steps in the Leloir pathway of galactose metabolism. — Intermediates and enzymes in the Leloir pathway of galactose metabolism<ref name="Leloir"/>

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.

References

(November 2003). "Structure and function of enzymes of the Leloir pathway for galactose metabolism". J. Biol. Chem..
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..

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metabolic-pathways inborn-errors-of-metabolism galactose

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