Methylcitrate was detected in 32(78%) patients, 3-OH isovalerate in 38(93%), 3-OH propionate 25(60%), tiglylglycine in 16(35%) and propionylglycine
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).