An intermediate in l-phenylalanine and l-tyrosine catabolism; accumulates in certain inherited disorders of tyrosine metabolism.
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DCA is primarily biotransformed to glyoxylate by the bifunctional enzyme glutathione transferase zetal and maleylacetoacetate isomerase (GSTzl/ MAAI), which also catalyzes the penultimate step in the phenylalanine and tyrosine catabolic pathway.
KEY WORDS: cancer, dichloroacetate, glutathione transferase zeta, hereditary tyrosinemia, maleylacetoacetate isomerase, mitochondrial disease, peripheral neuropathy, pyruvate dehydrogenase, toxicogenetics.
DCA is dehalogenated to glyoxylate by the cytosolic zeta-1 family isoform of glutathione transferase (GSTzl), which is identical to maleylacetoacetate isomerase (MAAI), the penultimate enzyme in the phenylalanine/tyrosine catabolic pathway [see Supplemental Material, Figure 2 (doi:10.
Genetic or DCA-induced ablation of GSTzl/MAAI causes accumulation of the MAAI substrates maleylacetone and, presumably, maleylacetoacetate (MAA) (Ammini et al.
Fernandez-Canon and Penalva (1998) have reported the Zeta GSTs in Aspergillus nidulans and humans are identical to an enzyme maleylacetoacetate isomerase (MAAI), which catalyzes the glutathione-dependent cis--trans isomerization of maleylacetoacetate to fumarylacetoacetate in the catabolic pathway of tyrosine/phenylalanine.
The first three-dimensional structure of a Zeta class GST has been characterized from Arabidopsis, which differs catalytically from previously characterised GSTs in that it adds glutathione reversibly to the cis double bond of maleylacetoacetate, allowing bond rotation before elimination of glutathione to yield fumarylacetoacetate, as those with Aspergillus nidulans and human (Thom et al.
Characterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue.
Maleylacetoacetate isomerase (MAAI/GSTZ)-deficient mice reveal a glutathione-dependent nonenzymatic bypass in tyrosine catabolism.
Intermediary metabolism and catabolism enzymes 2D3 6e-17 AY431429 Carboxypeptidase A Mosquito 1C1 2e-26 NP_918077 Putative carboxyme- thylenebmenolidase Rice 1F4 1e-38 XP_316948 Nudix * Mosquito 1F10 1e-76 CAE70680 Isocitrate dehydro- genase * Nematode 1H6 3e-27 XP_308198 Pancreatic lipase * Mosquito 15C3 2e-20 CAA93088 Giutathione S-trans- ferase * Nematode 15G3 9e-11 EAL31332 Maleylacetoacetate isomerase * Fly AVII.
GSTZ1d: a new allele of glutathione transferase zeta and maleylacetoacetate isomerase.
2004) reported that pretreatment with either DCA or TCE inhibits the cytosolic enzyme glutathione S-transferase (GST) [xi], also known as maleylacetoacetate (MAA) isomerase, and TCA pretreatment does not.