Three Types of Inborn Errors of Metabolism
Inborn Errors of Metabolism
Inborn errors of metabolism are rare genetic (inherited) disorders in which the body cannot properly turn food into energy. The disorders are usually caused by defects in specific proteins (enzymes) that help break down (metabolize) parts of food. Inborn errors of metabolism are a heterogeneous group of disorders that may be inherited or may occur as the result of spontaneous mutations. These diseases involve failure of the metabolic pathways involved in either the break-down or storage of carbohydrates, fatty acids, and proteins.
There are many different disorders of protein metabolism. A common factor in their management is restriction of natural dietary protein to avoid the accumulation of toxins in the body. Disorder-specific protein substitutes are used to ensure protein requirements are met.
Biovencer produces a comprehensive range of innovative and lifestyle-friendly protein substitutes for the various disorders. We have also developed support products for this area, which can typically be used in any of the protein disorders, to further help meet individual nutritional needs.
There are many different disorders of fat metabolism. These include long-chain fatty acid oxidation disorders (LC-FAOD). Dietary fat is a rich source of energy. The overwhelming majority of regular dietary fat in western diets is long-chain triglyceride (LCT). Individuals with an LC-FAOD cannot release the energy from LCT. Instead, LCT leads to the release of toxins. Replacing LCT with a different type of fat known as medium-chain triglyceride (MCT) provides an alternative form of energy and helps prevent toxin accumulation.
There are many different disorders of carbohydrate metabolism. Carbohydrate metabolism is a fundamental biochemical process that ensures a constant supply of energy to living cells. The most important carbohydrate is glucose, which can be broken down via glycolysis, enter into the Kreb's cycle and oxidative phosphorylation to generate ATP.