GSS - glutathione synthetase Gene

Homo sapiens

Also known as GSHS; HEL-S-64p; HEL-S-88n

Gene ID: 2937 | Gene type: protein coding

About GSS

Cytogenetic location: 20q11.22 Genomic coordinates (GRCh38): 20:34,928,432-34,956,027 (from NCBI)

This gene has 28 transcripts (splice variants), 245 orthologues and is associated with 4 phenotypes. Ubiquitous expression in kidney (RPKM 14.9), colon (RPKM 13.6) and 25 other tissues.

Summary

Glutathione is important for a variety of biological functions, including protection of cells from oxidative damage by free radicals, detoxification of xenobiotics, and membrane transport. The protein encoded by this gene functions as a homodimer to catalyze the second step of glutathione biosynthesis, which is the ATP-dependent conversion of gamma-L-glutamyl-L-cysteine to glutathione. Defects in this gene are a cause of glutathione synthetase deficiency. [provided by RefSeq, Jul 2008]

GSS Products(3)

mRNA	Protein	Name
NM_000178.4	NP_000169.1	glutathione synthetase
NM_001322494.1	NP_001309423.1	glutathione synthetase
NM_001322495.1	NP_001309424.1	glutathione synthetase

GSS Protein Structure

GSH_synth_ATP

GSH_synthase

0
100
200
300
400
474 a.a.

Protein Preferred Names

Protein Names

glutathione synthetase

GSH synthetase

Recombinant GSS Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70360	Glutathione synthetase/GSS Protein, Human (His)	P48637 (A2-V474)	≥95%

Related Diseases

Diseases

Alias

Glutathione Synthetase Deficiency

5-Oxoprolinuria	Pyroglutamic Aciduria
Pyroglutamicaciduria	Glutathione Synthetase Deficiency With 5-Oxoprolinuria
GSSD	Oxoprolinase Deficiency
5-Oxoprolinemia	Deficiency Of Glutathione Synthase
Deficiency Of Glutathione Synthetase	Pyroglutamic Acidemia
GSS DEFICIENCY	Gluthathione Synthetase Deficiency
5-Oxoprolinase Deficiency

Glutathione Synthetase Deficiency Of Erythrocytes, Hemolytic Anemia Due To

Hemolytic Anemia Due To Glutathione Synthetase Deficiency	GSSDE
Glutathione Synthetase Deficiency Of Erythrocytes	GLUSYNDE

Glutathione Synthetase Deficiency Of Erythrocytes

Glutathione Synthetase Deficiency Without 5-Oxoprolinuria

Metabolic Acidosis

Hemolytic Anemia

Anemia, Hemolytic	Anemia Hemolytic
Anaemia Due To Other Disorders Of Glutathione Metabolism	Chronic Non Spherocytic Anaemia
G6pd - [Glucose-6-Phosphate Dehydrogenase Deficiency] Anaemia	Anaemia Due To Glucose-6-Phosphate Dehydrogenase Deficiency
Glucose-6-Phosphate Dehydrogenase Deficiency With Anaemia	Glucose-6-Phosphate Dehydrogenase Deficiency Anaemia
Favism Anaemia	Haemolytic Anaemia Due Tog6pd Deficiency
Favism	Pentose Phosphate Pathway Disorder Anaemia
Anaemia Due To Pentose Phosphate Pathway Defect

Combined D-2- And L-2-Hydroxyglutaric Aciduria

D,L-2-Hydroxyglutaric Aciduria	D2L2AD
Combined D-2-Hydroxyglutaric Acidemia And L-2-Hydroxyglutaric Acidemia	Combined D-2-Hydroxyglutaric Aciduria And L-2-Hydroxyglutaric Aciduria
D,L-2-Hga	D,L-2-Hydroxyglutaric Acidemia
Combined D,L-2-Hydroxyglutaric Aciduria

2-Hydroxyglutaric Aciduria

2-Hga	2-Hydroxyglutaric Acidemia
2-Hydroxyglutaricaciduria	Combined D-2- And L-2-Hydroxyglutaric Aciduria

L-2-Hydroxyglutaric Aciduria

L-2-Hydroxyglutaric Acidemia	L2HGA
L-2-Hga	Aciduria, L-2-Hydroxyglutaric
Combined D-2- And L-2-Hydroxyglutaric Aciduria

D-2-Hydroxyglutaric Aciduria 1

D-2-Hydroxyglutaric Aciduria	D2HGA1
D-2-Hga	D-2-Hydroxyglutaric Acidemia
D2ha	D2hga
Aciduria, D-2-Hydroxyglutaric, Type 1	Combined D-2- And L-2-Hydroxyglutaric Aciduria

Cystinosis

Cystine Storage Disease	Cystine Diathesis
Cystine Disease	Cystinoses
Protein Defect Of Cystin Transport	Cystin Transport, Protein Defect Of
Nephropathic Cystinosis	Protein Defect Of Cystine Transport

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-RS05871	GSS Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Mus musculus	GSS	MGD	MGI:95852
Bos taurus	GSS	VGNC	VGNC:29683
Macaca mulatta	GSS	VGNC	VGNC:73301
Canis familiaris	GSS	VGNC	VGNC:41534
Rattus norvegicus	GSS	RGD	RGD:2752
Felis catus	GSS	VGNC	VGNC:67489
Others	GSS	NCBI

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