GCLC
GeneName
GCLC
Summary
GCLC, also known as gamma glutamylcysteine synthetase or GCS, is a 73 kDa enzyme primarily located in the cytosol and mitochondria. It plays a pivotal role in the biosynthesis of glutathione, a crucial antioxidant that helps maintain cellular redox homeostasis. GCLC is part of the glutamate-cysteine ligase complex and is involved in various metabolic processes, including cysteine and glutamate metabolism. The enzyme exhibits multiple binding activities, including ADP, ATP, and magnesium ion binding, which are essential for its function in cellular responses to various stimuli such as hormones and oxidative stress.
Importance
GCLC is relevant to: - The regulation of oxidative stress and redox balance, which is critical in preventing cellular damage and apoptosis. - Metabolic disorders associated with glutathione deficiency, impacting conditions like neurodegeneration and cancer. - The response mechanisms to environmental stressors, including heavy metals and xenobiotics, highlighting its role in detoxification. - Vascular health through its involvement in blood vessel diameter maintenance and response to growth factors, influencing angiogenesis and tissue repair.
Top Products
For researchers investigating GCLC, we highly recommend the top-selling recombinant antibody, Anti-GCLC antibody [EP13475] (ab190685). This antibody has been validated for use in Western blotting (WB) and immunoprecipitation (IP), making it a versatile tool for your experiments. With 71 citations, it is well-regarded in the research community, demonstrating its reliability and effectiveness in GCLC studies. This recombinant antibody offers the added benefit of batch-to-batch consistency, ensuring dependable results in your research. The Human GCLC ELISA Kit (ab233632), supported by 4 citations, is an excellent option for researchers looking to accurately measure GCLC levels in their samples.
Abcam Product Citation Summary
The data indicates that GCLC is being studied in various contexts related to oxidative stress, T cell activation, and cell differentiation. The use of Abcam antibody ab190685 in both mouse and human models highlights its relevance in understanding the role of GCLC in these biological processes, particularly in response to ischemia-reperfusion injury.
Abcam Product Citation Table
Function
Catalyzes the ATP-dependent ligation of L-glutamate and L-cysteine and participates in the first and rate-limiting step in glutathione biosynthesis.
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.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Sulfur metabolism; glutathione biosynthesis; glutathione from L-cysteine and L-glutamate: step 1/2.
Sequence Similarities
Belongs to the glutamate--cysteine ligase type 3 family.
Alternative names
GLCL, GLCLC, GCLC, Glutamate--cysteine ligase catalytic subunit, GCS heavy chain, Gamma-ECS, Gamma-glutamylcysteine synthetase