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%).
yeast cells grown in media supplemented with the iron chelator bathophenanthrolinedisulfonate (BPS) showed potential repression of the TCA cycle which is mediated by enzymes containing Fe/S clusters, reduced expression of enzymes involved in the glyoxylate pathway and methylcitrate
cycle, downregulation of the electron transport chain, and decreased in oxidative phosphorylation thus lowering ATP production.
This test reveals markedly elevated methylmalonic acid and elevated methylcitrate
, propionic acid, and 3-hydroxypropionic acid in patients with MMA.
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.
fumigatus relies more on the methylcitrate cycle (MCC) for survival in mammalian hosts .
Latge et al., "Methylcitrate synthase from Aspergillus fumigatus is essential for manifestation of invasive aspergillosis," Cellular Microbiology, vol.
and 3-hydroxy propionate, additional markers of propionic acidemia, were absent from urine organic acids, effectively ruling out defects in PCC.
One of these alternate pathways involves the condensation of propionyl-CoA with oxaloacetate to form methylcitrate
. This pathway uses enzymes of the Krebs cycle (5).
The PA cases were confirmed with the finding of increased urinary tiglylglycine, 3-hydroxypropionate, and methylcitrate
; the lymphocytes of both infants were without propionylcarboxylase activity.