2-Aminoadipate aminotransferase

I Homocitrate synthase 2 homocitrate dehydratase 3 homoaconitate hydratase 4 homoisocitrate dehydrogenase 5 2-aminoadipate aminotransferase 6 aminoadipate semialdehyde dehydrogenase 7 saccharopine dehydrogenase (L-glutamate forming) 8 saccharopine dehydrogenase (L-lysine forming)... 

If the outlined metabolic pathway is correct, 2-aminoadipic aciduria without or with minimal 2-oxoadipic aciduria could result from a defect in mitochondrial 2-aminoadipate aminotransferase deficiency with 2-oxoadi-pic aciduria only originating from tryptophan. A defect in 2-oxoadipate dehydrogenase would be the cause of 2-oxoadipic aciduria [6]. 

Table 12.3a+b. 2-Aminoadipate aminotransferase/2-oxoadipate dehydrogenase ia) (13 patients known) deficiency (2-amino/2- oxoadipic acidur-... 

Alkyldihydroxyacetonephosphate synthase deficiency 2-Aminoadipate aminotransferase deficiency 2-Aminoadipic semialdehyde synthetase deficiency -Aminolevulinic acid dehydratase deficiency 2-Amino-/2-oxoadipic aciduria Amylo-l,6-glucosidase deficiency Amylopectinosis Andersen disease Androgen Insensitivity syndrome Apolipoprotein A-I deficiency Apolipoprotein B-lOO deficiency Apolipoprotein C-II deficiency... 

This enzyme [EC 2.6.1.36], also called lysine e-aminotransferase, catalyzes the pyridoxal-phosphate-depen-dent reaction of L-lysine with a-ketoglutarate (or, 2-ox-oglutarate) to produce 2-aminoadipate 6-semialdehyde... 

Another laboratory-scale application for yield enhancement has been demonstrated in Streptomyces clavuligerus (72). Insertion of a second copy of the lot gene into the chromosome caused a two- to fivefold increase in production of lactam antibiotics- The lot gene encodes lysine e-aminotransferase, an enzyme involved in the biosynthesis of CX-aminoadipic acid, a precursor in penicillin and cephalosporin antibiotic biosynthesis. 

Figure 2 Lysine metabolism and its relationship to a aminoadipic acid production in bacteria atnl fungi. Abbreviation LAT, lysine e aminotransferase.

Coque JJR, Liras P, Laiz L, Martin JF. A gene encoding lysine 6-aminotransferase, which forms the 0-Iactam precursor a-aminoadipic acid, is located in the cluster of cephamycin biosynthetic gettes in Nocardki Idciumdurans. J Bacteriol 1991 173 6258-6264. 

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