A minor metabolite that accumulates in people with propionic acidemia.
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Methylcitrate was detected in 32(78%) patients, 3-OH isovalerate in 38(93%), 3-OH propionate 25(60%), tiglylglycine in 16(35%) and propionylglycine in 4(7.5%).
When propionyl-CoA accumulates, other derivative components are found in urine and blood, such as methylcitrate (formed by condensation of propionyl-CoA with oxaloacetate), propionylglycine, and propionylcarnitine.
2), which has several metabolic fates: [beta]-oxidation to form 3-hydroxypropionate, condensation with oxaloacetate to form 2-methylcitrate, conjugation with glycine to form propionylglycine, and conjugation with L-carnitine to form propionylcarnitine (C3).