MAAI Antibody (H-4): sc-374404

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In humans, maleylacetoacetate isomerase (MAAI, also known as GSTZ1-1) catalyzes the conversion of maleylacetoacetate to fumarylacetoacetate, the fifth step in the phenylalanine/phenylacetate degradation pathway (1-3). Deficiencies in other steps of this pathway cause metabolic diseases, including type I tyrosinemia and phenylketonuria (4,5). The glutathione S-transferases (GSTs) are found in all aerobic organisms and catalyze the conjugation of glutathione to a wide variety of electrophilic substrates (1). By sequence alignment and phylogenetic analysis, a new subgroup of GST-like proteins from human, C. elegans, and carnation were identified (2). Human MAAI is 38% and 49% identical to the carnation and C. elegans proteins, respectively (2). Recombinant human MAAI is a dimer. The enzyme exhibits limited activity with known GST substrates (2). Western blot analysis indicates that MAAI is most abundant in liver, with lower levels detected in skeletal muscle and brain (2). The gene which encodes MAAI maps to human chromosome 14q24.3 (3).