Glutaric Aciduria Type 1 (GA1)

Feeding your possible with GA1

What is GA1 (Glutaric Aciduria Type 1)?

Glutaric Aciduria Type 1, or GA1, is an inherited metabolic condition that is estimated to affect about one in every 100,000 people worldwide. A simple genetic change means that people with GA1 don’t produce enough of an enzyme called glutaryl-CoA dehydrogenase (GCDH), which is responsible for breaking down the amino acids lysine (Lys), hydroxylysine (Hyl) and tryptophan (Trp) in the protein that they eat. If people with GA1 eat too much protein, or break down more protein than usual (during illness, for example), glutaric acids (GAs), which are formed during Lys, Hyl and Trp breakdown, can build up in the body and cause problems. That’s why following a low protein diet is the way to reach your health potential and live your best life with GA1.

We call this “Feeding Your Possible”

Why should you feed your possible?

The gold standard in GA1 management is a low protein diet that includes a special nutritional supplement, L-carnitine supplements, and following a management plan during illness. If this regimen is started at a very early age and followed strictly, especially for the first six years of life, people diagnosed with GA1 can grow and develop to their full potential.

If GA1 is not well-managed, GAs can build up and cause damage to an area of the brain called the basal ganglia that controls movement. This can cause brain damage and permanently affect things like muscle control and swallowing, which can have a big impact on the way a person lives, and can even shorten life expectancy.

In adults and teenagers, a build up of GAs may still cause neurological problems and issues with movement, and scientists think it may play a role in other problems like kidney disease. Staying on diet for life is the way to be at your best if you have GA1.

How is GA1 diagnosed?

These days, all babies born in Australia and New Zealand are tested for GA1 a few days after birth, through the Newborn Screening Program. If the screening results show high levels of GAs, more tests are done to confirm a GA1 diagnosis. If GA1 is confirmed, a baby will immediately start on a special supplement to control GA levels. Starting diet and supplements early, and continuing through life is the best way to manage GA1.

How is GA1 managed?

Everyone has specific and individual dietary needs, whether they have GA1 or not, and reach their full potential by eating the food their bodies need. GA1 is really no different. Many people in Australia and New Zealand living with GA1 feed their possible by following an individualised diet that includes low protein food and a GA1 nutritional supplement, L-carnitine supplements, following an emergency treatment plan during illness, monitoring their GA levels, and regularly checking in with their metabolic team. When their condition is well-managed, people with GA1 live rich, fulfilling lives.

There are many ways you can feed your possible at Low Protein Connect. Get inspired by our low protein recipes, discover flavour and variety in the online store where you’ll find a wide range of GA1 supplements and low protein foods, and take time out for your wellbeing with our low protein lifestyle tips.

Reference sources:

Kölker S et al. J Inherit Metab Dis 2011;34(3):677–94.

National Organisation for Rare Diseases (NORD). Glutaric Aciduria Type I. Available at: https://rarediseases.org/rare-diseases/glutaricaciduria-i/. Accessed January 2021.

British Inherited Metabolic Diseases Group (BIMDG). TEMPLE (Tools Enabling Metabolic Patients Learning) GA1. Available at: https://bimdg.org.uk/store/temple//B20030_Temple_Updates_GA1_DIGITAL_-_Final_2020_299206_27112020.pdf. Accessed January 2021.

Genetic and Rare Diseases Information Center (GARD). Glutaric acidemia type 1. Available at: https://rarediseases.info.nih.gov/diseases/6522/glutaric-acidemia-type-i. Accessed January 2021.

Metabolic.ie National Centre for Inherited Metabolic Disorders. Glutaric Aciduria Type 1. Available at: https://metabolic.ie/wp-content/uploads/2015/06/GA-1-Handbook.pdf. Accessed January 2021.

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What is GA1? How is it diagnosed, what are the symptoms, and how does a low-protein diet help manage this condition?