fruktóz1foszáttá

Fruktóz1foszát, also known as fructose-1-phosphate, is a phosphorylated form of fructose. It is a key intermediate in the metabolism of fructose in both humans and other organisms. In the liver, fructose is primarily metabolized via the fructokinase pathway, where fructokinase catalyzes the phosphorylation of fructose to fructose-1-phosphate using adenosine triphosphate (ATP) as the phosphate donor. This initial step is crucial for trapping fructose within the cell and preparing it for further breakdown.

Following its formation, fructose-1-phosphate is then cleaved by aldolase B, another liver enzyme, into two triose phosphates: dihydroxyacetone phosphate (DHAP) and glyceraldehyde. These triose phosphates can then enter the glycolytic pathway, either to be converted into glucose or to be further oxidized for energy production.

Disruptions in fructose metabolism, particularly those affecting the enzymes involved in the fructose-1-phosphate pathway, can lead to genetic disorders. Hereditary fructose intolerance (HFI) is a classic example, caused by a deficiency in aldolase B. This deficiency leads to the accumulation of fructose-1-phosphate in the liver, which is toxic and can cause severe hypoglycemia, vomiting, and liver damage after ingestion of fructose or sucrose. Understanding the role of fruktóz1foszát is therefore essential for comprehending fructose metabolism and its associated metabolic diseases.