| T4766 |
3-Methylglutaric acid
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Endogenous Metabolite
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Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239 ). Methylglutaric acid is als... |
| T19340 |
Glutarylcarnitine
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O-glutaroyl-L-carnitine |
Endogenous Metabolite
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Glutarylcarnitine (O-glutaroyl-L-carnitine) is a diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes. |
| T5305 |
3-Methylbut-2-enoic acid
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3,3-Dimethylacrylic Acid,Senecioic acid,3-Methylcrotonic Acid |
Others
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Endogenous Metabolite
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3-Methylbut-2-enoic acid (Senecioic acid) appears in the urine of patients with 3-Methylcrotonic aciduria, which is a disorder characterized by urine that contains increased amounts of 3-methylcrotonic acid. |
| T5550 |
2-Hydroxy-2-methylbutanoic acid
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Others
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Endogenous Metabolite
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2-Hydroxy-2-methylbutyric acid is an unusual metabolite found in the urine of patients with 2-hydroxyglutaric aciduria and maple syrup urine disease. |
| T4861 |
Trans-Aconitic acid
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ATPase
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Endogenous Metabolite
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Trans-Aconitic acid is normally present in normal human urine, and it has been suggested that is present in larger amounts with Reye's syndrome and organic aciduria. trans-Aconitic acid is a substrate of enzyme trans-aco... |
| T5246 |
Trans-2-butene-1,4-dicarboxylic acid
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trans-2-Butene-1,4-dicarboxylic Acid,3-Hexenedioic Acid,trans-3-Hexenedioic Acid,β-Hydromuconic Acid |
Others
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Endogenous Metabolite
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Trans-2-butene-1,4-dicarboxylic acid (3-Hexenedioic Acid) is a normal human unsaturated dicarboxylic acid metabolite with increased excretion in patients with Dicarboxylic aciduria caused by fatty acid metabolism disorde... |
| T5273 |
2-Hydroxy-3-methylbutanoic acid
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2-Hydroxy-3-methylbutyric acid,2-Hydroxyisovaleric Acid,(±)-2-Hydroxyisopentanoic Acid |
Others
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Endogenous Metabolite
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2-Hydroxy-3-methylbutanoic acid ((±)-2-Hydroxyisopentanoic Acid) is a metabolite found in the urine of patients with phenylketonuria, methylmalonic acidemia, propionic acidemia, 3-ketothiolase deficiency, isovaleric acid... |
| T5232 |
3-Methyl-2-oxobutanoic acid
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2-Oxoisovaleric acid,alpha-Ketoisovaleric acid |
Others
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Endogenous Metabolite
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3-Methyl-2-oxobutanoic acid (2-Oxoisovaleric acid) is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 3-Methyl-2-oxobutanoic acid is a neurotoxin, an acidogen, and a metabo... |
| T4880 |
L-Dihydroorotic acid
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Others
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Endogenous Metabolite
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L-Dihydroorotic acid, also known as (S)-4,5-dihydroorotate or dihydro-L-orotate, belongs to the class of organic compounds known as alpha amino acids and derivatives. Within the cell, L-dihydroorotic acid is primarily lo... |
| T4901 |
5'-Guanylic acid disodium salt
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Guanosine 5'-monophosphate disodium salt,GMP-5 |
Others
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Endogenous Metabolite
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5'-Guanylic acid disodium salt (GMP-5) belongs to the class of organic compounds known as purine ribonucleoside monophosphates. In particular, L-Glutamic acid and guanosine monophosphate can be biosynthesized from xanthy... |
| T19289 |
DL-Glyceric Acid
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Glyceric Acid (20% in Water,ca.2 mol/L) |
Others
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DL-glyceric acid is a compound that is excessively secreted in the urine of patients with D-glyceric aciduria. |
| T19346 |
5'-Guanylic acid
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5'-guanosine monophosphate,5'-GMP |
Endogenous Metabolite
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5'-Guanylic acid (5'-guanosine monophosphate) is involved in several metabolic disorders, including AICA-ribosiduria pathway, adenosine deaminase deficiency,adenine phosphoribosyltransferase deficiency (aprt), and the 2-... |
| T38954 |
L-Glyceric acid sodium
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L-Glyceric acid sodium is a urinary metabolite predominantly found in individuals with the rare inherited metabolic disorder, L-glyceric aciduria. This compound serves as a diagnostic tool for identifying primary hyperox... |
