Aminosäurestoffwechselstörungen

Aminosäurestoffwechsel refers to the complex biochemical processes involved in the synthesis and degradation of amino acids within living organisms. Amino acids are the fundamental building blocks of proteins, and they also play crucial roles in various metabolic pathways, acting as precursors for neurotransmitters, hormones, and other vital molecules. The balance of amino acid metabolism is essential for maintaining cellular function and overall organismal health.

The synthesis of amino acids, known as anabolism, typically involves the transfer of nitrogen atoms from a source, often ammonia, to carbon skeletons. This process is often energy-intensive and requires specific enzymatic machinery. Organisms vary in their ability to synthesize essential amino acids; humans, for instance, can only synthesize a portion of the amino acids they require, while others must be obtained from the diet.

Conversely, the degradation of amino acids, or catabolism, occurs when excess amino acids are present or when the body needs to break them down for energy or to produce other metabolic intermediates. This process involves the removal of the amino group, which is then converted into urea or other nitrogenous waste products for excretion. The remaining carbon skeleton can be channeled into pathways like glycolysis, the citric acid cycle, or gluconeogenesis, depending on the specific amino acid. Disruptions in amino acid metabolism can lead to various metabolic disorders and diseases.