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CoFactor: Biotin

General information

2D representation

Biotin

Key facts

Cofactor type    prosthetic group
Human metabolism    Vitamin B7
IUPAC name    5-[(3aS,4S,6aR)-2-oxohexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoic acid
Curator    JDF

Tags

Molecular function

Biotin's main function as a cofactor is to transfer CO2 from one active site of an enzyme complex to another. It can also transfer C2-units [1].

Biotin further regulates gene expression, for instance by biotinylation of histones [1].

Chemical properties

In all cases, biotin is covalently bound to its partner enzyme and serves as a CO2-carrier between bicarbonate and the acceptor substrate [1].

Pathways

The cofactor is essential for fatty acid biosynthesis [1].

Comment

The biotin requirement is very low, so the only nutritional deficiency is found in patients with a diet rich in raw egg-white (avidin from raw egg white has a high affinity to biotin). Otherwise there are mainly genetic disorders, resulting from a loss of function of biotinidase (the recycling enzyme) or holocarboxylase sythetase (the biotin inserting enzyme) [1].

Part of the required biotin is produced by the intestinal flora [1]. Biotinidase recycles biotin from enzymes gained from food sources [1]. Organic acideria can result from multi-carboxylase deficiency [1]. Other resulting diseases include neurological disorders and developmental delay [1].

References

[1] pubmed:17898891
[2] pubmed:17898894
[3] pubmed:17898888
[4] pubmed:17898895
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