Hn Nh

Lys O BCCP Figure 28.40 • Coenzyme form of biotin.

the case of two identified genetic disorders. Holocarbo-xylase deficiency is a genetic disorder of biotin-(propionyl-CoA-carboxylase [ATP-hydrolysing]) ligase, the enzyme responsible for the terminal step incorporating biotin into the apoenzyme. Biotinidase (EC 3.5.1.12) deficiency is a genetic disorder of the amidase that releases biotin from the apoenzyme and allows it to be recycled. Both of these lead to multiple carboxylase deficiency (MCD), a condition marked by decreased activity of all biotin-dependent car-boxylases and both respond, at least partially, to large doses of biotin.197 The symptoms of biotin deficiency include dermatitis, conjunctivitis, alopecia, and CNS abnormalities including behavioral, developmental, and psychological effects.198

Was this article helpful?

0 0
How To Reduce Acne Scarring

How To Reduce Acne Scarring

Acne is a name that is famous in its own right, but for all of the wrong reasons. Most teenagers know, and dread, the very word, as it so prevalently wrecks havoc on their faces throughout their adolescent years.

Get My Free Ebook


Post a comment