1. Gene
  2. GSTZ1 - glutathione S-transferase zeta 1 Gene

GSTZ1 - glutathione S-transferase zeta 1 Gene

Homo sapiens

Also known as MAI; MAAI; MAAID; GSTZ1-1

Gene ID: 2954 | Gene type: protein coding

About GSTZ1

Cytogenetic location: 14q24.3 Genomic coordinates (GRCh38): 14:77,321,036-77,331,597 (from NCBI)

This gene has 19 transcripts (splice variants), 209 orthologues, 14 paralogues and is associated with 1 phenotype. Broad expression in liver (RPKM 23.8), testis (RPKM 10.9) and 24 other tissues.

Summary

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. [provided by RefSeq, Jul 2015]

GSTZ1 Products(4)

mRNA Protein Name
NM_001312660.2 NP_001299589.1 maleylacetoacetate isomerase isoform 3
NM_001363703.2 NP_001350632.1 maleylacetoacetate isomerase isoform 4
NM_145870.3 NP_665877.1 maleylacetoacetate isomerase isoform 1
NM_145871.3 NP_665878.2 maleylacetoacetate isomerase isoform 2
Gene Ontology
  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence Reference Source
enables glutathione transferase activity IDA
IDA: Inferred from direct assay
10739172 GOA
enables identical protein binding IPI
IPI: Inferred from physical interaction
16189514 GOA
enables maleylacetoacetate isomerase activity IDA
IDA: Inferred from direct assay
10739172 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
17474147 GOA
enables protein homodimerization activity IPI
IPI: Inferred from physical interaction
10739172 GOA
Biological Process GO Annotation Evidence Reference Source
involved in cellular detoxification IDA
IDA: Inferred from direct assay
20884751 GOA
involved in glutathione metabolic process IDA
IDA: Inferred from direct assay
10739172 GOA
Cellular Component GO Annotation Evidence Reference Source
is active in cytosol IDA
IDA: Inferred from direct assay
20884751 GOA
is active in mitochondrion IDA
IDA: Inferred from direct assay
20884751 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

GSTZ1 Protein Structure

GST_N_3

GST_N_3: Glutathione S-transferase, N-terminal domain (8 - 86)

GST_C

GST_C: Glutathione S-transferase, C-terminal domain (130 - 193)

  • 0
  • 100
  • 200
  • 216 a.a.
Protein Preferred Names Protein Names

maleylacetoacetate isomerase

S-(hydroxyalkyl)glutathione lyase

GSTZ1 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
GSTZ1 O43708 ZMYND12 Homo sapiens Q9H0C1 32296183
Intra
GSTZ1 O43708 ZMYND12 Homo sapiens Q9H0C1 32296183
Intra
GSTZ1 O43708 ZMYND12 Homo sapiens Q9H0C1 32296183
Intra
GSTZ1 O43708 CLVS2 Homo sapiens Q5SYC1 32296183
Intra
GSTZ1 O43708 CLVS2 Homo sapiens Q5SYC1 32296183
Intra
GSTZ1 O43708 CLVS2 Homo sapiens Q5SYC1 32296183
Intra
GSTZ1 O43708 TCP11 Homo sapiens Q8WWU5-7 32296183
Intra
GSTZ1 O43708 QARS1 Homo sapiens P47897 32296183
Intra
GSTZ1 O43708 TRAF2 Homo sapiens Q12933 32296183
Intra
GSTZ1 O43708 TRAF2 Homo sapiens Q12933 32296183
Intra
GSTZ1 O43708 TRAF2 Homo sapiens Q12933 32296183
Intra
GSTZ1 O43708 GORASP2 Homo sapiens Q9H8Y8 32296183
Intra
GSTZ1 O43708 GORASP2 Homo sapiens Q9H8Y8 32296183
Intra
GSTZ1 O43708 GORASP2 Homo sapiens Q9H8Y8 32296183
Intra
GSTZ1 O43708 GSTZ1 Homo sapiens O43708 32296183
Intra
GSTZ1 O43708 GSTZ1 Homo sapiens O43708 25416956
Intra
GSTZ1 O43708 PLEKHG4 Homo sapiens Q58EX7 32296183
Intra
GSTZ1 O43708 GSTZ1 Homo sapiens O43708 16189514
Intra
GSTZ1 O43708 PLEKHG4 Homo sapiens Q58EX7 32296183
Intra
GSTZ1 O43708 GSTZ1 Homo sapiens O43708 32296183
Intra
GSTZ1 O43708 PLEKHG4 Homo sapiens Q58EX7 32296183
Intra
GSTZ1 O43708 GSTZ1 Homo sapiens O43708 32296183
Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant GSTZ1 Proteins

Cat. No. Product Name Accession Purity
HY-P70206A Maleylacetoacetate isomerase/GSTZ1 Protein, Human (N-His) NP_665877.1 (Q2-A216) ≥95%

Related Diseases

Diseases Alias
Maleylacetoacetate Isomerase Deficiency

MAAID

Maai Deficiency

Hypersuccinylacetonemia, Mild

Mhsa

Benign Hypersuccinylacetonemia

Bhsa

Tyrosinemia, Type I

Tyrosinemia Type I

Hepatorenal Tyrosinemia

Fumarylacetoacetase Deficiency

Fah Deficiency

TYRSN1

Fumarylacetoacetate Hydrolase Deficiency

Tyrosinemia Type 1

Tyrosinemia 1

Fumarylacetoacetase

Tyrosinemia

Hypertyrosinemia

Tyrosinemias

Hereditary Tyrosinemia

Hypertyrosinaemia

Tyrosinaemia

Hereditary Hypertyrosinemia

Hawkinsinuria

4-Alpha-Hydroxyphenylpyruvate Hydroxylase Deficiency

4-Hppd Deficiency

4-Hydroxyphenylpyruvic Acid Dioxygenase Deficiency

HWKS

HAWK

Lactic Acidosis

Acidosis, Lactic

Acidosis Lactic

Alkaptonuria

Homogentisic Acid Oxidase Deficiency

Alcaptonuria

AKU

Deficiency Of Homogentisicase

Homogentisate 1,2-Dioxygenase Deficiency

Alkaptonuric Ochronosis

Homogentisic Acidura

Ochronosis, Hereditary

Hereditary Ochronosis

Ochronosis

Homogentisicaciduria

Deficiency Of Homogentisate Oxygenase

Tyrosinemia, Type Iii

Tyrosinemia Type Iii

4-Hydroxyphenylpyruvate Dioxygenase Deficiency

TYRSN3

4-Hydroxyphenylpyruvic Acid Oxidase Deficiency

Tyrosinemia Type 3

4-Alpha Hydroxyphenylpyruvate Dioxygenase Deficiency

4-Alpha Hydroxyphenylpyruvic Acid Oxidase Deficiency

Tyrosinemia Due To 4-Hydroxyphenylpyruvate Dioxygenase Deficiency

Tyrosinemia Due To 4-Hydroxyphenylpyruvic Acid Oxidase Deficiency

Tyrosinemia Due To Hpd Deficiency

Tyrosinemia 3

Tyrosinemia, Type Ii

Tyrosinemia Type Ii

Oculocutaneous Tyrosinemia

Richner-Hanhart Syndrome

Tyrosine Aminotransferase Deficiency

Tat Deficiency

Tyrosine Transaminase Deficiency

Keratosis Palmoplantaris With Corneal Dystrophy

TYRSN2

Oregon Type Tyrosinemia

Tyrosinemia Type 2

Tyrosinosis Oculocutaneous Type

Tyrosinosis, Oculocutaneous Type

Richner Hanhart Syndrome

Keratosis Palmoplantaris-Corneal Dystrophy Syndrome

Tyrosinemia Due To Tat Deficiency

Tyrosinemia Due To Tyrosine Aminotransferase Deficiency

Tyrosinemia 2

Tyrosinemia Oregon Type

Tyrosine Transaminase Deficiency Disease

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Felis catus GSTZ1 VGNC VGNC:67491
Mus musculus GSTZ1 MGD MGI:1341859
Macaca mulatta GSTZ1 VGNC VGNC:73304
Rattus norvegicus GSTZ1 RGD RGD:1589363
Bos taurus GSTZ1 VGNC VGNC:29688
Canis familiaris GSTZ1 VGNC VGNC:41537
Others GSTZ1 NCBI