Galactose mutarotase

Definition:

Mutarotase that catalyzes the interconversion of beta-D-galactose and alpha-D-galactose during galactose metabolism. Beta-D-galactose is metabolized in the liver into glucose 1-phosphate, the primary metabolic fuel, by the action of four enzymes that constitute the Leloir pathway: GALM, GALK1 (galactokinase), GALT (galactose-1-phosphate uridylyltransferase) and GALE (UDP-galactose-4'-epimerase). Involved in the maintenance of the equilibrium between the beta- and alpha-anomers of galactose, therefore ensuring a sufficient supply of the alpha-anomer for GALK1. Also active on D-glucose although shows a preference for galactose over glucose.

References:

[1]. David J Timson, et al. Identification and characterisation of human aldose 1-epimerase. FEBS Lett. 2003 May 22;543(1-3):21-4. [Content Brief]

[2]. Yoichi Wada, et al. Biallelic GALM pathogenic variants cause a novel type of galactosemia. Genet Med. 2019 Jun;21(6):1286-1294. [Content Brief]

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