Two different clinical disorders have been attributed to a defect of branched-chain amino acid transamination : hypervalinemia and hyperleucine-isoleucinemia.
22.
He is best known for his co-discovery, along with Maria Kritzman, of enzymatic transamination and its dependence on vitamin B6.
23.
The amino groups of amino acids are attached to it ( by transamination ) and carried to the liver where the urea cycle takes place.
24.
But some additional information is that this enzyme catalyzed it transamination with L isomer, but D isomer in natural form, inactive as substrate.
25.
Evidence suggests that they catalyze the transamination of GABA and the dehydrogenation of the product to form succinate, an intermediate in the Krebs cycle.
26.
Since transamination of proteins is a leading producer of ammonia, protein restriction may be recommended as a therapy to reduce the symptoms of the episode.
27.
This enzyme catalyzes the reversible transamination of branched-chain alpha-keto acids to branched-chain L-amino acids essential for cell growth.
28.
During the reaction, eflornithine's decarboxylation mechanism is analogous to that of ornithine in the active site, where transamination occurs with PLP followed by decarboxylation.
29.
But it was also shown that they can produce glutarate from delta-aminovalerate ( via transamination and dehydrogenation reactions ) as part of the degradation of lysine.
30.
Transamination in biochemistry is accomplished by enzymes called transaminases or aminotransferases . ?-ketoglutarate acts as the predominant aminogroup acceptor and produces glutamate as the new amino acid.