From the Research
Propionic acid, also known as Propia, is not a medication, but rather a compound that accumulates in patients with propionic acidemia, a rare metabolic disorder caused by a deficiency of propionyl-CoA carboxylase 1.
Definition and Context
Propionic acidemia is characterized by the inability to metabolize propionic acid, which combines with oxaloacetate to form methylcitric acid, leading to a deficiency in citric acid cycle intermediates.
Treatment and Management
The management of propionic acidemia involves anaplerotic therapy, which aims to fill up the citric acid cycle, and may include supplements such as citrate, glutamine, or ornithine α-ketoglutarate 1.
- Citrate supplementation has been shown to increase the urinary excretion of Krebs cycle intermediates, such as α-ketoglutarate, succinate, and fumarate, and may contribute to reducing hospitalizations in patients with propionic acidemia 1.
- L-carnitine supplementation has also been found to be effective in reducing toxic metabolites and oxidative damage in patients with propionic and methylmalonic acidemias 2, 3.
Key Considerations
The most recent and highest quality study suggests that L-carnitine treatment may have a protective role in reducing inflammatory and neurodegenerative biomarkers in patients with propionic and methylmalonic acidemias 2.
- Patients with propionic acidemia should be under the care of a healthcare provider to determine the best course of treatment and management, taking into account their specific situation and potential side effects.
- Regular follow-up appointments are recommended to monitor effectiveness and any potential side effects of treatment.