These proteins are related to the metal-dependent hydrolase superfamily [1]. The family includes 2-amino-3-carboxymuconate-6-semialdehyde decarboxylase which converts alpha-amino-beta-carboxymuconate-epsilon- semialdehyde (ACMS) to alpha-aminomuconate semialdehyde (AMS). ACMS can be converted non-enzymatically to quinolate, a potent endogenous excitoxin of neuronal cells which is implicated in the pathogenesis of various neurodegenerative disorders. In the presence of AMCSD, ACMS is converted to AMS, a benign catabolite.
Martynowski D, Eyobo Y, Li T, Yang K, Liu A, Zhang H.
Crystal structure of alpha-amino-beta-carboxymuconate-epsilon-semialdehyde decarboxylase: insight into the active site and catalytic mechanism of a novel decarboxylation reaction.
Biochemistry 45 2006 10412-21
[PubMed: 16939194] http://dx.doi.org/10.1021/bi060903q
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