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Glutathione biosynthesis involves two adenosine triphosphate-dependent steps: First, γ-glutamylcysteine is synthesized from L-glutamate and L-cysteine. This conversion requires the enzyme glutamate–cysteine ligase (GCL, glutamate cysteine synthase). This reaction is the rate-limiting step in glutathione synthesis. [3]
Glutathione synthetase (GSS) (EC 6.3.2.3) is the second enzyme in the glutathione (GSH) biosynthesis pathway. It catalyses the condensation of gamma-glutamylcysteine and glycine, to form glutathione. [2] Glutathione synthetase is also a potent antioxidant. It is found in many species including bacteria, yeast, mammals, and plants. [3]
Glutamate–cysteine ligase (GCL) EC 6.3.2.2), previously known as γ-glutamylcysteine synthetase (GCS), is the first enzyme of the cellular glutathione (GSH) biosynthetic pathway that catalyzes the chemical reaction:
Glutathione synthetase deficiency has an autosomal recessive pattern of inheritance. Mutations in the GSS gene cause glutathione synthetase deficiency. This gene provides instructions for making the enzyme glutathione synthetase. This enzyme is involved in a process called the gamma-glutamyl cycle, which takes place in most of the body's cells ...
14629 Ensembl ENSG00000001084 ENSMUSG00000032350 UniProt P48506 P97494 RefSeq (mRNA) NM_001498 NM_001197115 NM_010295 RefSeq (protein) NP_001184044 NP_001489 NP_034425 Location (UCSC) Chr 6: 53.5 – 53.62 Mb Chr 9: 77.66 – 77.7 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Glutamate–cysteine ligase catalytic subunit is an enzyme that in humans is encoded by the GCLC gene ...
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. Gamma glutamylcysteine synthetase deficiency has been implicated in some forms of hemolytic anemia. [6]
Buthionine sulfoximine (BSO) is a sulfoximine derivative which reduces levels of glutathione and is being investigated as an adjunct with chemotherapy in the treatment of cancer. [1] The compound inhibits gamma-glutamylcysteine synthetase, the enzyme required in the first step of glutathione synthesis.
Glutaredoxins function as electron carriers in the glutathione-dependent synthesis of deoxyribonucleotides by the enzyme ribonucleotide reductase. [6] Moreover, GRX act in antioxidant defense by reducing dehydroascorbate , peroxiredoxins , and methionine sulfoxide reductase.