L-gulonolactone oxidase

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gulonolactone (L-) oxidase pseudogene
Identifiers
SymbolGULOP
Entrez2989
HUGO4695
OMIM240400
RefSeqNG_001136
Other data
EC number1.1.3.8
LocusChr. 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.

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" (PDF). UK Food Standards Agency. Retrieved 2007-02-19.
  3. HYPOASCORBEMIA - NCBI
  4. OMIM - Online Mendelian Inheritance in Man - NCBI
  5. "Micronutrient intakes of wild primates: are humans different?" (PDF). Comp Biochem Physiol A Mol Integr Physiol. 2003 Sep;136(1):47-59. Retrieved 2007-03-11.

See also




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