The fact that CGMP is a peptide ensures that the absorption rate of its amino acids is prolonged compared to free amino acids and thereby results in improved protein retention and increased satiety compared to free amino acids. Another important benefit of CGMP is that the taste is significantly improved when CGMP substitutes part of the free amino acids and this may help ensure improved compliance to the PKU diet. Furthermore, CGMP contains a high amount of the Phe-lowering LNAAs, which constitutes about 41 g per 100 g protein and will therefore help maintain plasma phe levels in the target range. Enzyme substitutes
In 2018, the FDA approved an enzyme substitute called pegvaliase which metabolizes phenylalanine. It is for adults who are poorly managed on other treatments.Tetrahydrobiopterin (BH4) (a cofactor for the oxidation of phenylalanine) when taken by mouth can reduce blood levels of this amino acid in some people. Mothers
For women with PKU, it is important for the health of their children to maintain low Phe levels before and during pregnancy. Though the developing fetus may only be a carrier of the PKU gene, the intrauterine environment can have very high levels of phenylalanine, which can cross the placenta. The child may develop congenital heart disease, growth retardation, microcephaly and intellectual disability as a result.
Idarubicin  or 4-demethoxydaunorubicin is an anthracycline antileukemic drug. It inserts itself into DNA and prevents DNA unwinding by interfering with the enzyme topoisomerase II. It is an analog of daunorubicin, but the absence of a methoxy group increases its fat solubility and cellular uptake. Similar to other anthracyclines, it also induces histone eviction from chromatin.It belongs to the family of drugs called antitumor antibiotics. It is currently combined with cytosine arabinoside as a first line treatment of acute myeloid leukemia. It is used for treatment of acute lymphoblastic leukemia and chronic myelogenous leukemia in blast crisis.It is distributed under the trade names Zavedos (UK) and Idamycin (USA). Side effects
Diarrhea, stomach cramps, nausea and vomiting are common among patients treated with idarubicin. References
External links
Idarubicin bound to proteins in the PDB