Recombinant Human Maleylacetoacetate isomerase (GSTZ1)

Uniprot Id

O43708

Target Species

Human

Target Name

GSTZ1

Target Full Name

Maleylacetoacetate isomerase

Target Function

Bifunctional enzyme showing minimal glutathione-conjugating activity with ethacrynic acid and 7-chloro-4-nitrobenz-2-oxa-1,3-diazole and maleylacetoacetate isomerase activity. Has also low glutathione peroxidase activity with T-butyl and cumene hydroperoxides. Is able to catalyze the glutathione dependent oxygenation of dichloroacetic acid to glyoxylic acid.

Target Involvement

Maleylacetoacetate isomerase deficiency (MAAID)

Target Subcellular Location

Cytoplasm.

Target Protein Families

GST superfamily, Zeta family

Target Tissue Specificity

Mostly expressed in liver followed by kidney, skeletal muscle and brain. Also expressed in melanocytes, synovium, placenta, breast and fetal liver and heart.

Target Research Area

Signal Transduction

Target Synonyms

EC 2.5.1.18; EC 5.2.1.2; Glutathione S transferase zeta 1; Glutathione S-transferase zeta 1; Gstz1; GSTZ1-1; MAAI; MAAI_HUMAN; Maleylacetoacetate isomerase

Target Background

This gene is a member of the glutathione S-transferase (GSTs) super-family which encodes multifunctional enzymes important in the detoxification of electrophilic molecules, including carcinogens, mutagens, and several therapeutic drugs, by conjugation with glutathione. This enzyme catalyzes the conversion of maleylacetoacetate to fumarylacetoacatate, which is one of the steps in the phenylalanine/tyrosine degradation pathway. Deficiency of a similar gene in mouse causes oxidative stress. Several transcript variants of this gene encode multiple protein isoforms.