GSS - glutathione synthetase Gene

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

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 2937

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

Gene Ontology

Molecular Function

Molecular Function GO Annotation	Evidence	References	Source
enables ATP binding	IDA IDA: Inferred from direct assay	10369661	GOA
enables glutathione binding	IDA IDA: Inferred from direct assay	10369661	GOA
enables identical protein binding	IPI IPI: Inferred from physical interaction	21988832	GOA
enables magnesium ion binding	IDA IDA: Inferred from direct assay	10369661	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	21044950	GOA
enables protein homodimerization activity	IDA IDA: Inferred from direct assay	10369661	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

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

GSS Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Y2H Array	32296183
Intra	GSS	P48637	IRF3	Homo sapiens	Q14653	Anti Bait CoIP	18309294
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Y2H Prey Pooling	32296183
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Y2H	21988832
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Validated Y2H	26871637
Intra	GSS	P48637	TERF1	Homo sapiens	P54274	Pull Down	21044950
Intra	GSS	P48637	TERF1	Homo sapiens	P54274	BiFC	21044950
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Validated Y2H	32296183
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Y2H Array	25416956
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Y2H Prey Pooling	26871637
Intra	GSS	P48637	GSS	Homo sapiens	P48637	Y2H Array	26871637

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant GSS Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70360	Glutathione synthetase/GSS Protein, Human (His)	P48637-1 (A2-V474)	≥ 95%, as determined by reducing SDS-PAGE.

GSS Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P80411	Glutathione Synthetase Antibody (YA402)	WB, ICC/IF, IHC-P, FC	Human, Mouse, Rat

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

Pre-clinical Phase

Bioactive Molecules (7)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-113306	1-Methyladenine		99.99%	Unkown
HY-W015600	2-Acetamidophenol		99.73%	Unkown
HY-W015600S	2-Acetamidophenol-d3		99.48%	Unkown
HY-RS05871	GSS Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS11188	PRNP Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS17796	Gss Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS24264	Gss Rat Pre-designed siRNA Set A	Pre-designed Set	/	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