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L-gulonolactone oxidase
| gulonolactone (L-) oxidase pseudogene
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| Identifiers
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| Symbol
| GULOP
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| Entrez
| 2989
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| HUGO
| 4695
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| OMIM
| 240400
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| RefSeq
| NG_001136
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| Other data
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| EC number
| 1.1.3.8
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| Locus
| Chr. 8 p21.1
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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.
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.
See also
| Oxidoreductases: alcohol oxidoreductases (EC 1.1) |
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| 1.1.1 NAD/NADP acceptor | 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 |
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| 1.1.3 oxygen acceptor | Glucose oxidase - L-gulonolactone oxidase - Xanthine oxidase |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "L-gulonolactone_oxidase". A list of authors is available in Wikipedia.
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