L-gulonolactone oxidase

gulonolactone (L-) oxidase pseudogene
Identifiers
Symbol	GULOP
Entrez	2989
HUGO	4695
OMIM	240400
RefSeq	NG_001136
Other data
EC number	1.1.3.8
Locus	Chr. 8 p21.1

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

^ GULOP - iHOP
^ Vitamin C – Risk Assessment. UK Food Standards Agency. Retrieved on 2007-02-19.
^ HYPOASCORBEMIA - NCBI
^ OMIM - Online Mendelian Inheritance in Man - NCBI
^ Micronutrient intakes of wild primates: are humans different?. Retrieved on 2007-03-11.

Carbohydrate dehydrogenases - Alcohol dehydrogenase - Glycerol-3-phosphate dehydrogenase - L-xylulose reductase - Aldose reductase - Lactate dehydrogenase - 3-Hydroxyacyl CoA dehydrogenase - Malate dehydrogenase - Isocitrate dehydrogenase - Phosphogluconate dehydrogenase - Glucose-6-phosphate dehydrogenase - HMG-CoA reductase - Β-Ketoacyl ACP reductase - Hydroxysteroid dehydrogenase: 11 Beta (HSD11B1, HSD11B2) - 3 Beta (3-beta-HSD) - 17 Beta - Carnitine dehydrogenase - Beta-hydroxybutyryl-CoA dehydrogenase - IMP dehydrogenase - DXP reductoisomerase

1.1.3 oxygen acceptor

Glucose oxidase - L-gulonolactone oxidase - Xanthine oxidase

v • d • e Metabolism of vitamins, coenzymes, and cofactors
Folic acid	Dihydropteroate synthetase - Dihydrofolate reductase - Serine hydroxymethyltransferase
Vitamin D	25-Hydroxyvitamin D3 1-alpha-Hydroxylase
Thiamine	Thiamine pyrophosphokinase
Vitamin C	L-gulonolactone oxidase