dehydrogenase: a target and generator of oxidative stress.
2-Oxoglutarate (2-OG, also known as alpha-ketoglutarate
) is among these substances.
Huang et al., "Alpha-ketoglutarate
suppresses the NF-KB-mediated inflammatory pathway and enhances the PXR-regulated detoxification pathway," Oncotarget, vol.
Our patient with hyperglycemia and dysmorphic features had a deletion of 7.23 Mb comprising the region 7p13-p12.1, with involvement of 39 Online Mendelian Inheritance in Man genes, including glucokinase associated with maturity-onset diabetes of the young, CCM2 associated with type 2 cerebral cavernous malformations, insulin-like growth factors binding protein-3 associated with decreased postnatal growth, and oxoglutarate dehydrogenase associated with alpha-ketoglutarate
dehydrogenase deficiency (short stature, hypotonia, cognitive impairment, and movement abnormalities).
The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate
[sup] demonstrated that the ketogenic diet and Deanna Protocol (a diet consisting of 10% arginine alpha-ketoglutarate
, 1.0% gamma aminobutyric acid, 0.1% ubiquinol, 10% medium-chain triglyceride)-fed mice had a significantly longer survival time than the standard diet-fed mice.
Many studies report on the antioxidant properties of alpha-ketoglutarate
(AKG) which is an important intermediate in the Krebs cycle.
(AKG), an anion of alpha-ketoglutaric acid, is an important intermediate in the Krebs cycle, which couples amino acid metabolism with glucose oxidation.
BCAA-derived carbons can then be fed into the citric acid cycle to form alpha-ketoglutarate
, and then the alpha-ketoglutarate
can be transaminated to glutamate to maintain glutamate levels.
Glutamate dehydrogenation produces alpha-ketoglutarate
to replenish the tricarboxylic acid cycle intermediates.
Using GC-MS (Gas chromatography-mass spectrometry) to detect alpha-hydroxyisovalerate, lactate, pyruvate and alpha-ketoglutarate
. Random urine sample is usually sufficient.