ReviewMetabolic brain disease2014
α-Ketoglutaramate: an overlooked metabolite of glutamine and a biomarker for hepatic encephalopathy and inborn errors of the urea cycle.
Review in Metabolic brain disease, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
33 citing papers in PubMed, 85 citations in OpenAlex.
- The transaminase-ω-amidase pathway senses oxidative stress to control glutamine metabolism and α-ketoglutarate levels in endothelial cells.The EMBO journal · 2026Article
- The Impact of Metabolic Rewiring in Glioblastoma: The Immune Landscape and Therapeutic Strategies.International journal of molecular sciences · 2025Review
- The Balance of Ketoacids α-Ketoglutarate and α-Ketoglutaramate Reflects the Degree of the Development of Hepatoencephalopathy in Rats.International journal of molecular sciences · 2024Review
- Constraining activity and growth substrate of fungal decomposers via assimilation patterns of inorganic carbon and water into lipid biomarkers.Applied and environmental microbiology · 2024Article
- Energy Metabolites and Indicative Significance of α-Ketoglutarate and α-Ketoglutaramate in Assessing the Progression of Chronic Hepatoencephalopathy.Biomolecules · 2024Article
- Microbiota-gut-liver-brain axis and hepatic encephalopathy.Microbiome research reports · 2024Review
- Serum metabolomic signatures of patients with rare neurogenetic diseases: an insight into potential biomarkers and treatment targets.Frontiers in molecular neuroscience · 2024Article
- Definition of the Neurotoxicity-Associated Metabolic Signature Triggered by Berberine and Other Respiratory Chain Inhibitors.Antioxidants (Basel, Switzerland) · 2023Article
- Review
- Characterization of tea (Camellia sinensis L.) flower extract and insights into its antifungal susceptibilities of Aspergillus flavus.BMC complementary medicine and therapies · 2023Article
- Ferrous sulfate reverses cerebral metabolic abnormality induced by minimal hepatic encephalopathy.Metabolic brain disease · 2023Article
- Residual Amino Acid Imbalance in Rats during Recovery from Acute Thioacetamide-Induced Hepatic Encephalopathy Indicates Incomplete Healing.International journal of molecular sciences · 2023Article
- Tricarboxylic Acid Metabolite Imbalance in Rats with Acute Thioacetamide-Induced Hepatic Encephalopathy Indicates Incomplete Recovery.International journal of molecular sciences · 2023Article
- Maturation and culture affect the metabolomic profile of oocytes and follicular cells in young and old mares.Frontiers in cell and developmental biology · 2023Article
- Chromatomass-Spectrometric Method for the Quantitative Determination of Amino- and Carboxylic Acids in Biological Samples.Metabolites · 2022Article
- α-Ketoglutaramate-A key metabolite contributing to glutamine addiction in cancer cells.Frontiers in medicine · 2022Article
- Preparative Biocatalytic Synthesis of α-Ketoglutaramate.International journal of molecular sciences · 2021Article
- Metabolomic analysis of skeletal muscle before and after strenuous exercise to fatigue.Scientific reports · 2021Article
- Adaptive physiological water conservation explains hypertension and muscle catabolism in experimental chronic renal failure.Acta physiologica (Oxford, England) · 2021Article
- Glutamine metabolism in Th17/Treg cell fate: applications in Th17 cell-associated diseases.Science China. Life sciences · 2021Review
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 2 countries.
Funding
Abstract
Glutamine metabolism is generally regarded as proceeding via glutaminase-catalyzed hydrolysis to glutamate and ammonia, followed by conversion of glutamate to α-ketoglutarate catalyzed by glutamate dehydrogenase or by a glutamate-linked aminotransferase (transaminase). However, another pathway exists for the conversion of glutamine to α-ketoglutarate that is often overlooked, but is widely distributed in nature. This pathway, referred to as the glutaminase II pathway, consists of a glutamine transaminase coupled to ω-amidase. Transamination of glutamine results in formation of the corresponding α-keto acid, namely, α-ketoglutaramate (KGM). KGM is hydrolyzed by ω-amidase to α-ketoglutarate and ammonia. The net glutaminase II reaction is: L - Glutamine + α - keto acid + H2O → α - ketoglutarate + L - amino acid + ammonia. In this mini-review the biochemical importance of the glutaminase II pathway is summarized, with emphasis on the key component KGM. Forty years ago it was noted that the concentration of KGM is increased in the cerebrospinal fluid (CSF) of patients with hepatic encephalopathy (HE) and that the level of KGM in the CSF correlates well with the degree of encephalopathy. In more recent work, we have shown that KGM is markedly elevated in the urine of patients with inborn errors of the urea cycle. It is suggested that KGM may be a useful biomarker for many hyperammonemic diseases including hepatic encephalopathy, inborn errors of the urea cycle, citrin deficiency and lysinuric protein intolerance.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.