GCLC

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. The gene encoding the catalytic subunit encodes a protein of 367 amino acids with a calculated molecular weight of 72.773 kDa and maps to chromosome 6. The regulatory subunit is derived from a different gene located on chromosome 1p22-p21. Deficiency of gamma-glutamylcysteine synthetase in human is associated with enzymopathic hemolytic anemia. [provided by RefSeq]

Full Name

glutamate-cysteine ligase, catalytic subunit

Biological Process

Aging Source: Ensembl
Blood vessel diameter maintenance Source: UniProtKB
Cell redox homeostasis Source: UniProtKB
Cellular response to fibroblast growth factor stimulus Source: Ensembl
Cellular response to follicle-stimulating hormone stimulus Source: Ensembl
Cellular response to glucose stimulus Source: Ensembl
Cellular response to hepatocyte growth factor stimulus Source: Ensembl
Cellular response to insulin stimulus Source: Ensembl
Cellular response to mechanical stimulus Source: Ensembl
Cellular response to thyroxine stimulus Source: Ensembl
Cysteine metabolic process Source: UniProtKB
Glutamate metabolic process Source: UniProtKB
Glutathione biosynthetic process Source: UniProtKB
L-ascorbic acid metabolic process Source: Ensembl
Negative regulation of apoptotic process Source: UniProtKB
Negative regulation of extrinsic apoptotic signaling pathway Source: Ensembl
Negative regulation of hepatic stellate cell activation Source: Ensembl
Negative regulation of mitochondrial outer membrane permeabilization involved in apoptotic signaling pathway Source: Ensembl
Negative regulation of neuron apoptotic process Source: Ensembl
Negative regulation of protein ubiquitination Source: Ensembl
Negative regulation of transcription, DNA-templated Source: UniProtKB
Positive regulation of proteasomal ubiquitin-dependent protein catabolic process Source: Ensembl
Regulation of mitochondrial depolarization Source: Ensembl
Response to activity Source: Ensembl
Response to arsenic-containing substance Source: Ensembl
Response to cadmium ion Source: Ensembl
Response to heat Source: UniProtKB
Response to hormone Source: UniProtKB
Response to human chorionic gonadotropin Source: Ensembl
Response to interleukin-1 Source: Ensembl
Response to nitrosative stress Source: Ensembl
Response to nutrient Source: Ensembl
Response to oxidative stress Source: UniProtKB
Response to xenobiotic stimulus Source: Ensembl

Cellular Location

Cytosol; Mitochondrion; Glutamate-cysteine ligase complex

Involvement in disease

Hemolytic anemia due to gamma-glutamylcysteine synthetase deficiency (HAGGSD):
A disease characterized by hemolytic anemia, glutathione deficiency, myopathy, late-onset spinocerebellar degeneration, and peripheral neuropathy.