tiamiinipürofosfaat

Tiamiinipürofosfaat, also known as thiamine pyrophosphate (TPP), is the biologically active coenzyme form of thiamine (vitamin B1). It plays a crucial role in carbohydrate metabolism, acting as a cofactor for several key enzymes involved in energy production. Specifically, TPP is essential for the function of enzymes like pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and transketolase.

These enzymes are involved in vital metabolic pathways, including glycolysis, the citric acid cycle, and the pentose phosphate pathway. Pyruvate dehydrogenase converts pyruvate into acetyl-CoA, a molecule that enters the citric acid cycle. Alpha-ketoglutarate dehydrogenase is another important enzyme in the citric acid cycle. Transketolase, in the pentose phosphate pathway, is responsible for producing NADPH, a reducing agent vital for biosynthesis and antioxidant defense, and for generating precursors for nucleotide synthesis.

The deficiency of thiamine, and consequently TPP, leads to a condition known as beriberi. Beriberi can manifest in various forms, affecting the cardiovascular system (wet beriberi) or the nervous system (dry beriberi). Neurological symptoms can include confusion, memory loss, and coordination problems. The widespread importance of TPP in cellular energy production highlights the critical role of thiamine in maintaining overall health and proper physiological function.