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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.

Additional recommended knowledge

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

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

See also

 
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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