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Identification
HMDB Protein ID HMDBP00817
Secondary Accession Numbers
  • 6097
  • HMDBP06196
Name Maleylacetoacetate isomerase
Synonyms
  1. GSTZ1-1
  2. Glutathione S-transferase zeta 1
  3. MAAI
Gene Name GSTZ1
Protein Type Enzyme
Biological Properties
General Function Involved in catalytic activity
Specific 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.
Pathways
  • Alkaptonuria
  • Disulfiram Action Pathway
  • Dopamine beta-hydroxylase deficiency
  • Hawkinsinuria
  • L-phenylalanine degradation
  • Monoamine oxidase-a deficiency (MAO-A)
  • Phenylalanine and Tyrosine Metabolism
  • Phenylketonuria
  • Tyrosine Metabolism
  • Tyrosine metabolism
  • Tyrosinemia Type 2 (or Richner-Hanhart syndrome)
  • Tyrosinemia Type 3 (TYRO3)
  • Tyrosinemia Type I
  • Tyrosinemia, transient, of the newborn
Reactions
Maleylacetoacetic acid → 4-Fumarylacetoacetic acid details
RX + Glutathione → HX + R-S-glutathione details
GO Classification
Biological Process
glutathione metabolic process
L-phenylalanine catabolic process
tyrosine catabolic process
Cellular Component
cytosol
mitochondrion
Component
cell part
intracellular part
cytoplasm
Function
catalytic activity
Molecular Function
glutathione peroxidase activity
glutathione transferase activity
maleylacetoacetate isomerase activity
Process
metabolic process
cellular metabolic process
cellular amino acid and derivative metabolic process
cellular amino acid metabolic process
aromatic amino acid family metabolic process
Cellular Location
  1. Cytoplasm
Gene Properties
Chromosome Location 14
Locus 14q24.3
SNPs GSTZ1
Gene Sequence
>651 bp
ATGCAGGCGGGGAAGCCCATCCTCTATTCCTATTTCCGAAGCTCCTGCTCATGGAGAGTT
CGAATTGCTCTGGCCTTGAAAGGCATCGACTACGAGACGGTGCCCATCAATCTCATAAAG
GATGGGGGCCAACAGTTTTCTAAGGACTTCCAGGCACTGAATCCTATGAAGCAGGTGCCA
ACCCTGAAGATTGATGGAATCACCATTCACCAGTCACTGGCCATCATTGAGTATCTAGAG
GAGATGCGTCCCACTCCGCGACTTCTGCCTCAGGACCCAAAGAAGAGGGCCAGCGTGCGT
ATGATTTCTGACCTCATCGCTGGTGGCATCCAGCCCCTGCAGAACCTGTCTGTCCTGAAG
CAAGTGGGAGAGGAGATGCAGCTGACCTGGGCCCAGAACGCCATCACTTGTGGCTTTAAC
GCCCTGGAGCAGATCCTACAGAGCACAGCGGGCATATACTGTGTAGGAGACGAGGTGACC
ATGGCTGATCTGTGCTTGGTGCCTCAGGTGGCAAATGCTGAAAGATTCAAGGTGGATCTC
ACCCCCTACCCTACCATCAGCTCCATCAACAAGAGGCTGCTGGTCTTGGAGGCCTTCCAG
GTGTCTCACCCCTGCCGGCAGCCAGATACACCCACTGAGCTGAGGGCCTAG
Protein Properties
Number of Residues 216
Molecular Weight 17895.68
Theoretical pI 5.954
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Maleylacetoacetate isomerase
MQAGKPILYSYFRSSCSWRVRIALALKGIDYKTVPINLIKDRGQQFSKDFQALNPMKQVP
TLKIDGITIHQSLAIIEYLEEMRPTPRLLPQDPKKRASVRMISDLIAGGIQPLQNLSVLK
QVGEEMQLTWAQNAITCGFNALEQILQSTAGIYCVGDEVTMADLCLVPQVANAERFKVDL
TPYPTISSINKRLLVLEAFQVSHPCRQPDTPTELRA
GenBank ID Protein 7417477
UniProtKB/Swiss-Prot ID O43708
UniProtKB/Swiss-Prot Entry Name MAAI_HUMAN
PDB IDs
GenBank Gene ID AC007954
GeneCard ID GSTZ1
GenAtlas ID GSTZ1
HGNC ID HGNC:4643
References
General References
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  3. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
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  5. Fernandez-Canon JM, Penalva MA: Characterization of a fungal maleylacetoacetate isomerase gene and identification of its human homologue. J Biol Chem. 1998 Jan 2;273(1):329-37. [PubMed:9417084 ]
  6. Board PG, Baker RT, Chelvanayagam G, Jermiin LS: Zeta, a novel class of glutathione transferases in a range of species from plants to humans. Biochem J. 1997 Dec 15;328 ( Pt 3):929-35. [PubMed:9396740 ]
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  8. Fernandez-Canon JM, Hejna J, Reifsteck C, Olson S, Grompe M: Gene structure, chromosomal location, and expression pattern of maleylacetoacetate isomerase. Genomics. 1999 Jun 15;58(3):263-9. [PubMed:10373324 ]
  9. Tong Z, Board PG, Anders MW: Glutathione transferase zeta catalyses the oxygenation of the carcinogen dichloroacetic acid to glyoxylic acid. Biochem J. 1998 Apr 15;331 ( Pt 2):371-4. [PubMed:9531472 ]
  10. Blackburn AC, Tzeng HF, Anders MW, Board PG: Discovery of a functional polymorphism in human glutathione transferase zeta by expressed sequence tag database analysis. Pharmacogenetics. 2000 Feb;10(1):49-57. [PubMed:10739172 ]
  11. Polekhina G, Board PG, Blackburn AC, Parker MW: Crystal structure of maleylacetoacetate isomerase/glutathione transferase zeta reveals the molecular basis for its remarkable catalytic promiscuity. Biochemistry. 2001 Feb 13;40(6):1567-76. [PubMed:11327815 ]