Circulating levels of 8-leucine are elevated in the cobalamin-deficient state of pernicious anemia. Levels of leucine, on the other hand, are much lower. It is proposed that leucine 2,3-aminomutase, the cobalamindependent enzyme that catalyzes the interconversion of leucine and 8-leucine, is the affected enzyme in pernicious anemia and causes these results by preventing the synthesis of leucine...

Mechanistic and structural studies on methylmalonyl-CoA mutase Swinging arms in multifunctional enzymes and the specificity of post-translational modification S-Adenosylmethionine: a ‘poor man’s coenzyme B,2’ in the reaction of lysine 2,3-aminomutase Enzymes that exploit imines one way or the other Preparation and properties of 6-substituted cyclohexaneI ,2,4-triol derivatives: mechanistic prob...

Pancytopenia in childhood can be caused by a variety of underlying diseases including hematological and non-hematological entities. Phenylketonuria (PKU) is an inborn error of phenylalanine metabolism. No association between PKU and pancytopenia has ever been reported. We report the first case of PKU revealed by a pancytopenia at presentation. The patient was an infant girl born to healthy non-...

cloning and expression of the l-phenylalanine dehydrogenase gene, from b. sphaericus in e. coli were done. the gene was cloned in the vector pet16b and transformed into e. coli bl21 (de3). the functional form of the l-phenylalanine dehydrogenase enzyme was purified by affinity purification techniques, taking advantage of the ability of this enzyme to bind to the nucleotide site affinity dye, re...

Phenylalanine ammonia-lyase (PAL) links the plant primary and secondary metabolisms, its product, trans-cinnamic acid, is derived into thousands of diverse phenylpropanoids. Bambusa oldhamii BoPAL4 has broad substrate specificity using L-phenylalanine, L-tyrosine, L-3,4-dihydroxy phenylalanine (L-DOPA) as substrates to yield p-coumaric caffeic respectively. The optimum reaction pH for three was...

Trans-cinnamic acid and p-coumaric are valuable intermediates in the synthesis of flavonoids widely employed food, flavor pharmaceutical industries. These products can be produced by deamination L-phenylalanine L-tyrosine catalyzed phenylalanine ammonia lyase or tyrosine lyase. Phenylalanine ammonia-lyase (PAL, EC 4.3.1.5) from Rhodotorula glutinis do not exhibit strong substrate specificity co...