Review Article
Mass Spectrometry-Based Metabolomic and Proteomic Strategies in Organic Acidemias
Table 3
Organic acids and acylglycines in OAs.
| | | Urinary organic acids detected by GC-MS | Urinary acylglycines detected by LC-MS/MS |
| | MSUD | 2-Keto-isocaproic acid ↑ | | | 2-OH-isovaleric acid ↑ | | | 2-Keto-isovaleric acid ↑ | | | 2-Keto-3-methylvaleric acid ↑ | | | 2-OH-3-methylvaleric acid ↑ | |
| | PA | 3-OH-propionic acid ↑ | Tiglylglycine ↑ | | Methylcitric acid ↑ | Propionylglycine ↑ |
| | MMA | Methylmalonic acid ↑ | | | Methylcitric acid ↑ | |
| | IVA | 3-Hydroxyisovaleric acid ↑ | Isovalerylglycine ↑ |
| | GA I | Glutaric acid ↑ | Glutarylglycine ↑ | | 3-OH glutaric acid ↑ |
| | β-KTD | 2-Methyl-3-OH butyric acid ↑ | Tiglylglycine ↑ | | 2-Methyl-acetoacetic acid ↑ |
| | HMG-CoA lyase D | 3-Methyl-glutaconic acid ↑ | |
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↑ means increase.
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