Glutaminase kidney isoform, mitochondrial
Also known as: GLS, GLS1, KIAA0838
Function
Catalyzes the first reaction in the primary pathway for the renal catabolism of glutamine. Plays a role in maintaining acid-base homeostasis. Regulates the levels of the neurotransmitter glutamate, the main excitatory neurotransmitter in the brain.
Classification
- Family (Pfam)
- PF12796 Ank_2, PF17959 EF-hand_14, PF04960 Glutaminase
- InterPro
- Ankyrin_rpt, Ankyrin_rpt-contain_sf, Beta-lactam/transpept-like, Glutaminase, Glutaminase_EF-hand
- Functional cluster
- Mixed Regulatory & Membrane Proteins
Experimental structures · PDB · 50
- 3CZD X-ray 2.40A
- 3UNW X-ray 2.56A
- 3UO9 X-ray 2.30A
- 3VOY X-ray 2.20A
- 3VOZ X-ray 2.40A
- 3VP0 X-ray 2.40A
- 3VP1 X-ray 2.30A
- 3VP2 X-ray 2.70A
- 3VP3 X-ray 2.70A
- 3VP4 X-ray 2.45A
- 4O7D X-ray 2.30A
- 5D3O X-ray 2.79A
- … and 38 more
A predicted model is available from AlphaFold.
Gene Ontology · 8
Disease associations
Drugs targeting this protein · 1
- TELAGLENASTAT inhibitor
Related proteins · sequence + function similarity
- Glutaminase kidney isoform, mitochondrial 1.00
- Glutaminase kidney isoform, mitochondrial 1.00
- Phospholipase ABHD3 0.75
- WD repeat-containing protein 26 0.72
- Sodium/hydrogen exchanger 8 0.72
- WD repeat-containing protein 26 0.72
- Elongator complex protein 3 0.72
- Elongator complex protein 3 0.72
- Phospholipase ABHD3 0.72
- WD repeat-containing protein 26 0.71
- WD repeat-containing protein 26 0.71
- WD repeat-containing protein 26 0.71
Co-cited proteins · studied together in the literature
- Glutaminase liver isoform, mitochondrial 1 shared papers
- Glutaminase kidney isoform, mitochondrial 2 shared papers
- Glutaminase kidney isoform, mitochondrial 2 shared papers
- Glutaminase liver isoform, mitochondrial 1 shared papers
- Dual specificity mitogen-activated protein kinase kinase 2 1 shared papers
- Caytaxin 1 shared papers
- Caytaxin 1 shared papers
- Caytaxin 1 shared papers
- RAF proto-oncogene serine/threonine-protein kinase 1 shared papers
Literature · 21 cited papers
- Glutaminase deficiency caused by short tandem repeat expansion in GLS. N. Engl. J. Med. · 2019
- Identification of a loss-of-function mutation in the context of glutaminase deficiency and neonatal epileptic encephalopathy. JAMA Neurol. · 2019
- GLS hyperactivity causes glutamate excess, infantile cataract and profound developmental delay. Hum. Mol. Genet. · 2019
- Characterization of the interactions of potent allosteric inhibitors with glutaminase C, a key enzyme in cancer cell glutamine metabolism. J. Biol. Chem. · 2018
- The origin and evolution of human glutaminases and their atypical C-terminal ankyrin repeats. J. Biol. Chem. · 2017
- Design and evaluation of novel glutaminase inhibitors. Bioorg. Med. Chem. · 2016
- N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
- Structural basis for the active site inhibition mechanism of human kidney-type glutaminase (KGA). Sci. Rep. · 2014
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- Structural basis for the allosteric inhibitory mechanism of human kidney-type glutaminase (KGA) and its regulation by Raf-Mek-Erk signaling in cancer cell metabolism. Proc. Natl. Acad. Sci. U.S.A. · 2012
- Mitochondrial localization and structure-based phosphate activation mechanism of glutaminase C with implications for cancer metabolism. Proc. Natl. Acad. Sci. U.S.A. · 2012
- Full-length human glutaminase in complex with an allosteric inhibitor. Biochemistry · 2011
- … and 9 more in the literature graph
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