Transamination from Phe yields phenylpyruvic acid
, of which as much as 1-2 g/day may be excreted in the urine.
Nitisinone (A drug) inhibits 4-hydroxy phenylpyruvic acid
dioxygenase as will be discussed in treatment options.
According to (http://www.nature.com/tp/journal/v3/n1/full/tp2012143a.html#bib63) Nature , hydrogen sulfide can be toxic to the mitochondria when it interacts synergistically with phenylpyruvic acid
(PPA) commonly found in stools.
Antidiabetic enolic glucoside of phenylpyruvic acid
. PCT Patent Application No.
, indole3-carbinol, and glycocholic acid positively correlate with the B2 (p_Cyanobacteria;f_ unassigned), B5 (p_Firmicutes;f_unassigned), and B7 (p_Firmicutes;f_Clostridiaceae) families, respectively, whereas dihydrodaidzein negatively correlates with the B8 family (p_Firmicutes;f_Clostridiales Family XIII Incertae Sedis).
They have a specific function such as pyruvic acid (PYR) is involved in the biosynthesis of alanine [22-23] while the concentration of phenylpyruvic acid
(PPY) is increased in the plasma of patients with hereditary metabolic disease [24-25] and it inhibits renal gluconeogenesis .
The lack of PAH leads to hyperphenylalanemia and ultimately to metabolism of phenylalanine by an alternate pathway that results in the buildup of this alternate pathway metabolite called phenylpyruvic acid
in the blood.
Determination of [alpha]-keto acids including phenylpyruvic acid
in human plasma by high-performance liquid chromatography with chemiluminescence detection.
These are parahydroxy phenylactic acid and parahydroxy phenylpyruvic acid
. In addition, succinylacetone and succinylacetoacetate are found in the urine.