#1 Clinical Trials Search in Europe

Oxogluric Acid

This article explores a clinical trial investigating the use of oxogluric acid (also known as alpha-ketoglutarate) and other nutritional supplements in treating propionic acidemia. The study aims to determine if these supplements can improve hyperammonemia, glutamine levels, and overall outcomes in patients with this rare metabolic disorder.

Table of Contents

What is OXOGLURIC ACID?

OXOGLURIC ACID, also known as ornithine alpha-ketoglutarate (OKG), is a nutritional supplement that is being studied as a potential treatment for a rare genetic disorder called propionic acidemia[1]. It’s important to note that this substance is not yet approved as a medication for this condition, but is undergoing clinical trials to determine its safety and effectiveness.

Understanding Propionic Acidemia

Propionic acidemia is a rare genetic disorder that affects the body’s ability to break down certain proteins and fats[1]. This condition is caused by a deficiency of an enzyme called propionyl CoA carboxylase. As a result, people with this disorder can’t properly process some parts of proteins and fats, leading to a build-up of harmful substances in the body.

One of the main problems in propionic acidemia is hyperammonemia, which means there’s too much ammonia in the blood. This can be very dangerous and cause serious health problems, including brain damage if not treated promptly[1].

How OXOGLURIC ACID May Work

Researchers believe that OXOGLURIC ACID might help patients with propionic acidemia by supporting a crucial process in the body called the citric acid cycle (also known as the Krebs cycle)[1]. This cycle is essential for producing energy in our cells.

In propionic acidemia, there’s a shortage of important substances needed for this cycle to work properly. OXOGLURIC ACID might help by providing some of these missing substances, potentially improving the body’s ability to produce energy and handle ammonia better[1].

Clinical Trial Information

A clinical trial (NCT00645879) is being conducted to study the effects of OXOGLURIC ACID and other similar substances in patients with propionic acidemia[1]. The trial aims to see if these supplements can help reduce hyperammonemia and improve overall health outcomes for patients.

Dosage and Administration

In the clinical trial, OXOGLURIC ACID is being tested at a dose of 400 mg/kg of body weight, up to a maximum of 16 grams per day. This dose is divided into two parts and taken twice a day for 4 weeks[1]. If it’s found to be the most effective treatment, it may be continued for an additional 30 weeks.

It’s important to note that this dosage is specific to the clinical trial and should not be attempted without medical supervision.

Potential Benefits

The researchers hope that OXOGLURIC ACID might help in several ways[1]:

  • Reducing hyperammonemia (high levels of ammonia in the blood)
  • Improving levels of important amino acids like glutamine and glutamate
  • Enhancing overall energy production in the body
  • Potentially improving developmental outcomes and reducing medical complications

Other Treatments Being Studied

In addition to OXOGLURIC ACID, the clinical trial is also studying two other substances that might help patients with propionic acidemia[1]:

  1. Glutamine: This is an amino acid that’s important for many bodily functions. It’s being tested at the same dose as OXOGLURIC ACID.
  2. Disodium citrate (also known as sodium citrate): This is a form of citric acid. It’s being tested at a dose of 7.5 mEq/kg or 658 mg/kg, up to a maximum of 16 grams per day.

These substances are all being studied to see which one might be most effective in helping patients with propionic acidemia[1].

Aspect Details
Study Title Anaplerotic Therapy in Propionic Acidemia
Primary Objective To determine if nutritional supplements can improve hyperammonemia and outcomes in propionic acidemia patients
Supplements Tested Ornithine alpha-ketoglutarate, Glutamine, Disodium citrate
Dosage 400 mg/kg up to 16 g per day, split into 2 doses
Trial Duration 4 weeks for each supplement, followed by washout periods; best supplement tested for additional 30 weeks
Expected Outcomes Improved plasma glutamine levels, fewer episodes of hyperammonemia, better developmental outcomes
Potential Impact New approach to treating severe metabolic disorders by replacing downstream products involved in energy generation

FAQ

  • What is propionic acidemia? Propionic acidemia is a rare genetic disorder caused by a deficiency of the enzyme propionyl CoA carboxylase. This deficiency impairs the body's ability to break down certain proteins and fats, leading to a buildup of toxic substances in the blood.
  • What is the main goal of this clinical trial? The main goal is to determine if nutritional supplements like oxogluric acid (alpha-ketoglutarate), glutamine, or citrate can improve hyperammonemia (high ammonia levels in the blood), glutamine levels, and overall outcomes in patients with propionic acidemia.
  • How are these supplements administered in the trial? The supplements are mixed with formula or other foods and given in two daily doses. The initial trial period for each supplement is 4 weeks, followed by a washout period. The most effective supplement will then be tested for an additional 30 weeks.
  • What are the potential benefits of this treatment approach? This approach aims to fill up the citric acid cycle, potentially improving energy production in cells. If successful, it could lead to fewer episodes of hyperammonemia, better developmental outcomes, and improved overall health for patients with propionic acidemia.
  • How is this treatment different from current therapies for propionic acidemia? Current therapies focus on restricting certain dietary components and administering carnitine. This new approach aims to replace downstream products involved in energy generation, which could potentially be more effective in preventing long-term complications of the disease.

Ongoing Clinical Trials on Oxogluric Acid

  • Study comparing Custodiol-N and Custodiol organ preservation solutions for heart transplantation in children

    Recruiting

    2 1 1 1
    Investigated diseases:
    Investigated drugs:
    Germany

  • Study on Organ Preservation in Kidney, Liver, and Pancreas Transplants Using Custodiol-N Solution Compared to a Drug Combination for Transplant Patients

    Recruiting

    3 1 1 1
    Investigated diseases:
    Investigated drugs:
    Austria

  • Study Comparing Custodiol-N and Custodiol for Heart Surgery in Children with Congenital Heart Defects

    Not yet recruiting

    2 1 1 1
    Investigated diseases:
    Investigated drugs:
    Germany

  • Study on the Effects of Custodiol and Buckberg Cardioplegia in Patients Undergoing Major Heart Surgery with Prolonged Aortic Clamping

    Not yet recruiting

    3 1 1 1
    Investigated drugs:
    Spain

  • Study on Liver Transplantation: Comparing Custodiol-N Solution with a Drug Combination for Organ Preservation in Patients Undergoing Liver Transplant Surgery

    Not recruiting

    3 1 1 1
    Investigated diseases:
    Investigated drugs:
    Spain

Glossary

  • Propionic acidemia: A rare genetic disorder caused by a deficiency of the enzyme propionyl CoA carboxylase, leading to an accumulation of toxic substances in the blood.
  • Hyperammonemia: A condition characterized by high levels of ammonia in the blood, which can be toxic to the brain and other organs.
  • Anaplerotic therapy: A treatment approach that aims to replenish intermediates of metabolic cycles, in this case, the citric acid cycle.
  • Citric acid (Krebs) cycle: A series of chemical reactions in cells that generate energy from the breakdown of nutrients.
  • Alpha-ketoglutarate: An important molecule in the citric acid cycle, also known as oxogluric acid.
  • Glutamine: An amino acid that plays a role in various bodily functions, including protein synthesis and acid-base balance.
  • Citrate: A salt of citric acid, which is an intermediate in the citric acid cycle.
  • ATP: Adenosine triphosphate, the primary energy currency of cells.
  • Washout period: A period during a clinical trial when participants stop taking the study medication to allow its effects to wear off before starting the next phase.

References

  1. https://clinicaltrials.gov/study/NCT00645879