GLUL
GeneName
GLUL
Summary
GLUL, also known as glutamine synthetase or p42, is a 42 kDa enzyme that plays a pivotal role in the synthesis of glutamine from glutamate and ammonia. It is predominantly expressed in the brain and is found in various cellular compartments including the cytoplasm, mitochondria, and the nucleus. This enzyme is crucial for maintaining nitrogen balance and intracellular ammonium homeostasis, and it is involved in processes such as glutamate catabolism and glutamine biosynthesis. GLUL also participates in several biological pathways including angiogenesis and the regulation of endothelial cell migration, highlighting its importance in both metabolic and signalling pathways.
Importance
GLUL is relevant to: - Neurobiology, particularly in the context of neurotransmitter metabolism and the regulation of synaptic function - Cancer research, as it influences tumour microenvironment and cell proliferation - Metabolic disorders, given its role in nitrogen metabolism and response to starvation - Vascular biology, through its involvement in angiogenesis and endothelial cell regulation
Top Products
For researchers investigating GLUL, we recommend two excellent primary antibodies. The first is the well-cited polyclonal antibody, Anti-Glutamine Synthetase antibody (ab73593), which has garnered 110 citations, highlighting its reliability in the field. This antibody is suitable for immunohistochemistry (IHC), western blotting (WB), and immunocytochemistry (ICC), making it a versatile choice for various applications.Additionally, we offer the recombinant antibody, Anti-Glutamine Synthetase antibody [EPR13022(B)] (ab176562). This monoclonal antibody has been validated in knockout models and is effective in IHC and WB. With 32 citations, it is gaining traction among researchers. The recombinant nature of this product ensures batch-to-batch consistency, making it an excellent option for those requiring dependable GLUL detection. The Human Glutamine Synthase ELISA Kit (ab288927) is an excellent option for researchers looking to measure GLUL levels in their samples.
Abcam Product Citation Summary
The data indicates that GLUL antibodies have been effectively used in various western blotting experiments across different species, including mouse, rat, and human cell lines. The studies focus on contexts such as liver tissue, diabetes progression, and cancer cell lines, highlighting the relevance of GLUL in metabolic and pathological processes.
Abcam Product Citation Table
Developmental stage
Expressed during early fetal stages.
Function
Glutamine synthetase that catalyzes the ATP-dependent conversion of glutamate and ammonia to glutamine (PubMed:16267323, PubMed:30158707, PubMed:36289327). Its role depends on tissue localization: in the brain, it regulates the levels of toxic ammonia and converts neurotoxic glutamate to harmless glutamine, whereas in the liver, it is one of the enzymes responsible for the removal of ammonia (By similarity). Essential for proliferation of fetal skin fibroblasts (PubMed:18662667). Independently of its glutamine synthetase activity, required for endothelial cell migration during vascular development: acts by regulating membrane localization and activation of the GTPase RHOJ, possibly by promoting RHOJ palmitoylation (PubMed:30158707). May act as a palmitoyltransferase for RHOJ: able to autopalmitoylate and then transfer the palmitoyl group to RHOJ (PubMed:30158707). Plays a role in ribosomal 40S subunit biogenesis (PubMed:26711351). Through the interaction with BEST2, inhibits BEST2 channel activity by affecting the gating at the aperture in the absence of intracellular L-glutamate, but sensitizes BEST2 to intracellular L-glutamate, which promotes the opening of BEST2 and thus relieves its inhibitory effect on BEST2 (PubMed:36289327).
Involvement in disease
Glutamine deficiency, congenital
GLND
An autosomal recessive disorder characterized by variable brain malformations, encephalopathy, severe developmental delay, seizures, and decreased glutamine levels in bodily fluids. Death in early infancy may occur.
None
The disease is caused by variants affecting the gene represented in this entry.
Developmental and epileptic encephalopathy 116
DEE116
A form of epileptic encephalopathy, a heterogeneous group of early-onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. DEE116 is autosomal dominant form characterized by severe developmental delay, seizures, and white matter abnormalities.
None
The disease is caused by variants affecting the gene represented in this entry. DEE116 is caused by variants that disrupt the canonical translation start codon in GLUL resulting in initiation of translation at Met-18 (PubMed:38579670). The resulting protein is enzymatically competent but insensitive to negative feedback regulation via glutamine-induced degradation.
Post-translational modifications
Acetylated by EP300/p300; acetylation is stimulated by increased glutamine levels and promotes ubiquitin-mediated proteasomal degradation.
Palmitoylated; undergoes autopalmitoylation.
Ubiquitinated by ZNRF1 (By similarity). Ubiquitinated by the DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex called CRL4(CRBN), leading to proteasomal degradation (PubMed:26990986).
Sequence Similarities
Belongs to the glutamine synthetase family.
Tissue Specificity
Expressed in endothelial cells.
Cellular localization
- Cytoplasm
- Cytosol
- Microsome
- Mitochondrion
- Cell membrane
- Lipid-anchor
- Mainly localizes in the cytosol, with a fraction associated with the cell membrane.
Alternative names
GLNS, GLUL, Glutamine synthetase, GS, Glutamate--ammonia ligase, Palmitoyltransferase GLUL