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Anaplerotic therapy in propionic acidemia

Anaplerotic therapy in propionic acidemia

ANAPLEROTIC THERAPY IN PROPIONIC ACIDEMIA - PMC - NCBI

The objective of this study was to determine whether supplements with glutamine (400 mg/kg per day), citrate (7.5 mEq/kg per day), or ornithine α-ketoglutarate ...

Anaplerotic therapy in propionic acidemia - PubMed

These results indicate that citrate entered the Krebs cycle providing successful anaplerotic therapy by increasing levels of the downstream intermediates of ...

Anaplerotic therapy in propionic acidemia - ScienceDirect.com

These results indicate that citrate entered the Krebs cycle providing successful anaplerotic therapy by increasing levels of the downstream intermediates of ...

Anaplerotic therapy in propionic acidemia | Institutional Repository ...

Propionic acidemia is a rare metabolic disorder caused by a deficiency of propionyl- CoA carboxylase, the enzyme that converts propionyl-CoA to ...

Biochemical and anaplerotic applications of in vitro models of ...

3.1.2. Propionate treatment increases TCA cycle intermediates, but shows some evidence of TCA inhibition. Propionic acid, P-CoA, and its metabolites have been ...

Figure 1 from Anaplerotic therapy in propionic acidemia.

Figure 1. Propionyl-CoA and glutamine metabolism in propionic acidemia. Propionyl-CoA is carboxylated by propionyl CoA carboxylase to become ...

Riverside Clinical Trial Anaplerotic Therapy in Propionic Acidemia

Anaplerotic Therapy in Propionic Acidemia ... Propionic acidemia is caused by deficiency of propionyl CoA carboxylase that impairs the supply of succinyl CoA to ...

Anaplerotic therapy in propionic acidemia | Request PDF

Patients with propionic acidemia cannot metabolize propionic acid, which combines with oxaloacetate to form methylcitric acid. This, with the defective supply ...

Anaplerotic agents for treatment of disorders of propionate and long ...

Methods of treating propionic acidemia (PA), methylmalonic acidemia (MMA) and fatty acid oxidation disorders are described.

Sodium citrate Completed Phase 1 Trials for Propionic Acidemia (PA ...

NCT00645879. Anaplerotic Therapy in Propionic Acidemia. Benzalkonium (DB11105) · Citric acid (DB04272) · Glutamic acid (DB00142) · L-Glutamine (DB00130) ...

Understanding molecular mechanisms in propionic acidemia and ...

anaplerotic therapy · antioxidants · antisense oligonucleotides · ataluren · propionic acidemia · readthrough drugs · RNA therapies. Declaration ...

Citric acid Completed Phase 1 Trials for Propionic Acidemia (PA ...

NCT00645879. Anaplerotic Therapy in Propionic Acidemia. Benzalkonium (DB11105) · Citric acid (DB04272) · Glutamic acid (DB00142) · L-Glutamine (DB00130) ...

Interorgan amino acid interchange in propionic acidemia

Most efforts in the treatment of PA up to now have been aimed to avoid hyperammonemia episodes, which are considered the marker of a good or bad ...

Cardiac Complications in Patients with Propionic Acidemia

The usual treatment of long chain FAOs is with medium chain triglyceride oil, which can bypass the metabolic block, but doesn't address the ...

Anaplerotic Therapy in Propionic Acidemia – CheckOrphan

Anaplerotic Therapy in Propionic Acidemia · Recruitment Information · Administrative Informations · Related News · Related Clinical Trial · Related · About ...

Anaplerotic therapy in propionic acidemia

Background: Propionic acidemia is a rare metabolic disorder caused by a deficiency of propionyl- CoA carboxylase, the enzyme converting propionyl-CoA to ...

Anaplerotic diet therapy in inherited metabolic disease

Information is provided regarding the inter-organ requirements for more normal metabolic function during crisis and how anaplerotic therapy using ...

Pathophysiology of propionic and methylmalonic acidemias. Part 2 ...

Despite realizing increased survival rates for propionic acidemia (PA) and methylmalonic acidemia (MMA) patients, the current therapeutic ...

Fasting alleviates metabolic alterations in mice with propionyl-CoA ...

Propionic acidemia (PA), resulting from Pcca or Pccb gene mutations, impairs propionyl-CoA metabolism and induces metabolic alterations.

Genetics of Propionic Acidemia (Propionyl CoA Carboxylase ...

In 1961, Childs et al published the earliest clinical report of a patient who was ultimately found to be affected by a deficiency of ...