Alpha-aminoadipic semialdehyde synthase, mitochondrial

Definition:

Bifunctional enzyme that catalyzes the first two steps in lysine degradation.

References:

[1]. J Dancis, et al. Familial hyperlysinemia: enzyme studies, diagnostic methods, comments on terminology. Am J Hum Genet. 1979 May;31(3):290-9. [Content Brief]

[2]. K A Sacksteder, et al. Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia. Am J Hum Genet. 2000 Jun;66(6):1736-43. [Content Brief]

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