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Chemical compound
α-Hydroxyglutaric acid (2-hydroxyglutaric acid) is an alpha hydroxy acid form of glutaric acid. In humans the compound is formed by a hydroxyacid-oxoacid
Α-Hydroxyglutaric_acid
Medical condition
2-hydroxyglutaric aciduria is a rare neurometabolic disorder characterized by the significantly elevated levels of hydroxyglutaric acid in one's urine
2-Hydroxyglutaric_aciduria
Metabolic enzyme PHGDH
phosphohydroxypyruvic acid (i.e. 3-phosphonooxypyruvic acid), reduced NADH, and a proton. This enzyme can also catalyse the interconversion of α-hydroxyglutaric acid and
Phosphoglycerate dehydrogenase
Phosphoglycerate_dehydrogenase
Medical condition
acids lysine, hydroxylysine and tryptophan. Excessive levels of their intermediate breakdown products (glutaric acid, glutaryl-CoA, 3-hydroxyglutaric
Glutaric_aciduria_type_1
Class of enzymes
catalyzes the chemical reaction (S)-2-Hydroxyglutaric acid + electron acceptor α-Ketoglutaric acid + reduced acceptor The two substrates
L-2-hydroxyglutarate dehydrogenase
L-2-hydroxyglutarate_dehydrogenase
Dog breed
which appear in the breed include hydroxyglutaric aciduria, which is where elevated levels of Alpha-Hydroxyglutaric acid are in the dog's urine, blood plasma
West_Highland_White_Terrier
Organic compound with two –COOH groups
a dicarboxylic acid is an organic compound containing two carboxyl groups (−COOH). The general molecular formula for dicarboxylic acids can be written
Dicarboxylic_acid
Index of chemical compounds with the same molecular formula
148.12 g/mol, exact mass: 148.0372 u) may refer to: Citramalic acid α-Hydroxyglutaric acid This set index page lists chemical structure articles associated
C5H8O5
Class of enzymes
catalyzes the chemical reaction (R)-2-Hydroxyglutaric acid + electron acceptor α-Ketoglutaric acid + reduced acceptor The two substrates
D-2-hydroxyglutarate dehydrogenase
D-2-hydroxyglutarate_dehydrogenase
reaction (S)-3-hydroxybutyric acid + α-ketoglutaric acid acetoacetic acid + (R)-2-hydroxyglutaric acid The two substrates of this enzyme
Hydroxyacid-oxoacid transhydrogenase
Hydroxyacid-oxoacid_transhydrogenase
Class of enzymes
Glyoxylic acid + Propanoyl-CoA H2O α-Hydroxyglutaric acid + Coenzyme A The three substrates of this enzyme are glyoxylic acid, propanoyl-CoA
2-hydroxyglutarate_synthase
Protein-coding gene in the species Homo sapiens
into L-2-hydroxyglutaric aciduria: mass isotopomer studies reveal 2-oxoglutaric acid as the metabolic precursor of L-2-hydroxyglutaric acid". Journal
L2HGDH
dihydroxyheptanoic acid unit and a ring system with different substituents. The statin pharmacophore is modified hydroxyglutaric acid component, which is
Discovery and development of statins
Discovery_and_development_of_statins
Specialized technique associated with MRI
discovery that it can be used to probe the concentration of alpha-Hydroxyglutaric acid, which is only present in IDH1 and IDH2 mutated gliomas, which alters
In vivo magnetic resonance spectroscopy
In_vivo_magnetic_resonance_spectroscopy
Chemical compound
dialdehyde. Glutaric, 3-hydroxyglutaric, and glutaconic acids are structurally related metabolites. In glutaric aciduria type 1, glutaconic acid accumulates, resulting
Glutaconic_acid
Chemical compound
is associated with elevated levels of organic acids, including glutaric acid and 3-hydroxyglutaric acid . Glutaryl-CoA dehydrogenase Rao KS, Albro M,
Glutaryl-CoA
Class of enzymes
includes arginine 41 making a salt bridge to the carboxylate of the hydroxyglutaric acid closest to metal ion. A mutation in Arg 41 results in a drastic decrease
3-Hydroxy-3-methylglutaryl-CoA lyase
3-Hydroxy-3-methylglutaryl-CoA_lyase
Protein-coding gene in the species Homo sapiens
the formation and accumulation of the metabolites glutaric acid and 3-hydroxyglutaric acid as well as glutarylcarnitine in body fluids, which essentially
Glutaryl-CoA_dehydrogenase
Chemical compound
hydroxyglutamic acid terminus. There can be 1–5 units. In (tetrahydro)sarcinapterin (H 4SPT), a glutamyl group is linked to the 2-hydroxyglutaric acid terminus
Tetrahydromethanopterin
Protein-coding gene in the species Homo sapiens
Medicine. Stellmer F, Keyser B, Burckhardt BC, et al. (July 2007). "3-Hydroxyglutaric acid is transported via the sodium-dependent dicarboxylate transporter
Organic_anion_transporter_1
Class of genetic diseases
Disorders of organic acid metabolism (organic acidurias) alkaptonuria Combined malonic and methylmalonic aciduria (CMAMMA) 2-hydroxyglutaric acidurias Disorders
Inborn_errors_of_metabolism
Protein-coding gene in humans
2-hydroxyglutaric aciduria, a condition that causes progressive damage to the brain. The major types of this disorder are called D-2-hydroxyglutaric aciduria
IDH2
Class of enzymes
aldolase, 2-keto-4-hydroxyglutaric aldolase, 4-hydroxy-2-ketoglutarate aldolase, 2-keto-4-hydroxyglutarate aldolase, 2-oxo-4-hydroxyglutaric aldolase,
4-hydroxy-2-oxoglutarate aldolase
4-hydroxy-2-oxoglutarate_aldolase
Mammalian protein found in Homo sapiens
L-2-hydroxyglutaric aciduria, which was the first reported case of a pathogenic mutation of the SLC25A1 gene. Patients with D-2/L-2-hydroxyglutaric aciduria
Tricarboxylate transport protein, mitochondrial
Tricarboxylate_transport_protein,_mitochondrial
IUPAC Nomenclature of a catalyst enzyme
of Escherichia coli K-12 and its possible implications for human 2-hydroxyglutaric aciduria". Journal of Bacteriology. 178 (1): 232–9. doi:10.1128/jb
Phosphoserine_transaminase
InterPro Family
of Escherichia coli K-12 and its possible implications for human 2-hydroxyglutaric aciduria". J. Bacteriol. 178 (1): 232–9. doi:10.1128/jb.178.1.232-239
4-phosphoerythronate dehydrogenase
4-phosphoerythronate_dehydrogenase
Rare metabolic disorder
hereditary optic neuropathy (LHON), glutaric aciduria type 1, D-2-hydroxyglutaric aciduria, biotin-thiamine-responsive basal ganglia disease, Huntington
MEPAN_syndrome
enzyme in central metabolism, and its accumulation in humans causes L-2-Hydroxyglutaric aciduria. The repair enzyme L-2-hydroxyglutarate dehydrogenase oxidizes
Metabolite_damage
Protein found in humans
detects somatic mutations of IDH1 in metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria (MC-HGA)". American Journal of Medical Genetics. Part A. 155A
Isocitrate_dehydrogenase_1
220100; SLC7A9 Cytochrome C oxidase deficiency; 220110; COX6B1 D-2-hydroxyglutaric aciduria; 600721; D2HGDH Dandy–Walker malformation; 220200; ZIC1 Dandy–Walker
List_of_OMIM_disorder_codes
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID
Girl/Female
Latin
Named for Venus.
Male
English
 English form of Welsh Kai, KAY means "lord." In Arthurian legend, this is the name of one of the first Knights of the Round Table. He was the son of Sir Ector, the foster brother of King Arthur, and is noted for having an acid tongue and boorish behavior, but mostly for trying to take credit when Arthur pulled the sword from the stone. Compare with another form of Kay.Â
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID
HYDROXYGLUTARIC ACID