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

GSTZ1 Protein Structure

GST_N_3

GST_C

0
100
200
216 a.a.

Protein Preferred Names

Protein Names

maleylacetoacetate isomerase

S-(hydroxyalkyl)glutathione lyase

Recombinant GSTZ1 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70206	Maleylacetoacetate isomerase/GSTZ1 Protein, Human (C-His)	O43708 (M1-A216)	≥95%
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

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS05889	GSTZ1 Human Pre-designed siRNA Set A		/	Unkown

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

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