Hepatic fructokinase

Ketohexokinase (CHD ) (also: Hepatic fructokinase ) the name of the enzyme, the fructose is phosphorylated. This is the first step in the utilization of fructose by vertebrates. In human coronary artery disease is localized above average in liver, kidney, intestine, spleen and pancreas. Mutations in the CHD gene can lead fructosuria, the hereditary inability to digest fructose to the ( rare ) - it is excreted in the urine in this case.

There are two isoforms of CHD, called A and C, which arise from alternative splicing. C isoform is localized mainly in the liver. Active CHD is a dimer composed of one or both isoforms. There is probably just a lack of isoform C in fructosuria.

Catalyzed reaction

ATP ADP

β -D-fructose is phosphorylated to fructose -1-phosphate. As more substrates D -sorbose, D- Agatose or 5- Dehydrofructose are possible. The reaction is stimulated by the presence of potassium and decelerated by ADP.

Protein isoform Human Genome Organisation Euteleostomi Adenosine diphosphate
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