L-gulonolactone oxidase

L-gulonolactone oxidase (EC 1.1.3.8) is an enzyme that catalyzes the reaction of D-glucuronolactone (also known as L-gulono-1,4-lactone) with oxygen to L-xylo-hex-3-ulonolactone and hydrogen peroxide. It uses FAD as a cofactor.

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The CAS number for this enzyme is 9028-78-8

Gulonolactone oxidase deficiency

The non-functional GULOP was mapped to human chromosome 8p21 that corresponds to an evolutionarily conserved segment on either porcine chromosome 4 (SSC4) or 14 (SSC14).^[1] GULO produces ascorbic acid, which is often called "vitamin C".

Animals that have lost the ability to synthesize vitamin C are, notably: simians (primates), guinea pigs, the red-vented bulbul, fruit-eating bats and a species of trout.^[2]

Gulonolactone oxidase deficiency is called "hypoascorbemia"^[3] and is described by OMIM (Online Mendelian Inheritance in Man)^[4] as "a public inborn error of metabolism", as it affects all humans. There exists a wide discrepancy between the amounts of ascorbic acid other primates consume and what is recommended as "reference intakes" for humans.^[5]

References

See also

• Vitamin C
• ascorbic acid
• Oxidoreductase
• scurvy