NP_001184044.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
72,766 Da
NCBI Official Full Name
glutamate--cysteine ligase catalytic subunit isoform b
NCBI Official Synonym Full Names
glutamate-cysteine ligase, catalytic subunit
NCBI Protein Information
glutamate--cysteine ligase catalytic subunit; GCS heavy chain; gamma-ECS; gamma-glutamylcysteine synthetase
UniProt Protein Name
Glutamate--cysteine ligase catalytic subunit
UniProt Synonym Protein Names
GCS heavy chain; Gamma-ECS; Gamma-glutamylcysteine synthetase
UniProt Synonym Gene Names
UniProt Entry Name
GSH1_HUMAN
NCBI Summary for gamma-ECS
Glutamate-cysteine ligase, also known as gamma-glutamylcysteine synthetase is the first rate-limiting enzyme of glutathione synthesis. The enzyme consists of two subunits, a heavy catalytic subunit and a light regulatory subunit. This locus encodes the catalytic subunit, while the regulatory subunit is derived from a different gene located on chromosome 1p22-p21. Mutations at this locus have been associated with hemolytic anemia due to deficiency of gamma-glutamylcysteine synthetase and susceptibility to myocardial infarction.[provided by RefSeq, Oct 2010]
UniProt Comments for gamma-ECS
GCLC: Defects in GCLC are the cause of hemolytic anemia (HAGGSD). Belongs to the glutamate--cysteine ligase type 3 family.
Protein type: Ligase; EC 6.3.2.2; Other Amino Acids Metabolism - glutathione
Chromosomal Location of Human Ortholog: 6p12
Cellular Component: glutamate-cysteine ligase complex; cytosol
Molecular Function: glutamate binding; protein heterodimerization activity; glutamate-cysteine ligase activity; magnesium ion binding; coenzyme binding; ADP binding; ATP binding
Biological Process: glutamate metabolic process; response to arsenic; L-ascorbic acid metabolic process; response to hormone stimulus; response to nitrosative stress; cysteine metabolic process; apoptotic mitochondrial changes; cell redox homeostasis; sulfur amino acid metabolic process; response to heat; positive regulation of proteasomal ubiquitin-dependent protein catabolic process; xenobiotic metabolic process; glutathione biosynthetic process; negative regulation of protein ubiquitination; negative regulation of neuron apoptosis; response to oxidative stress; regulation of mitochondrial depolarization; regulation of blood vessel size; negative regulation of transcription, DNA-dependent; negative regulation of apoptosis
Disease: Gamma-glutamylcysteine Synthetase Deficiency, Hemolytic Anemia Due To; Myocardial Infarction, Susceptibility To
Research Articles on gamma-ECS
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Products associated with gamma-ECS elisa kit
Pathways associated with gamma-ECS elisa kit
Diseases associated with gamma-ECS elisa kit
Organs/Tissues associated with gamma-ECS elisa kit
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