What happens with HPU?
With HPU, the enzymes that produce the haem in the body do not work properly. In most HPU patients, 3 to 4 out of 8 haem-producing enzymes are impaired in their performance. As a result, the body sometimes produces incorrectly folded haem, which cannot function properly due to its defective structure. This affects:
the oxygen supply of the organs via the blood
the oxygen supply of the muscles
the energy production of the cells
HPU patients also have correctly folded haem, otherwise they would not be viable at all. The wrongly assembled haem, however, has another effect on the body:
Loss of important vital nutrients
The wrongly folded haem is toxic to the body. The body binds it to vitamin B6, zinc and partly also manganese in order to be able to excrete it better. This makes the complex water-soluble and the misfolded haem in HPU patients can be excreted via the kidneys and urine. During this process, the body loses these important nutrients.
Since vitamin B6 and zinc are co-factors for over 300 human enzymes, it quickly becomes clear that a lack of supply with these vital substances can lead to a variety of symptoms. Manganese is also needed for an important enzyme: superoxide dismutase (SOD) in the mitochondria. It protects the mitochondria from free radicals and has a detoxifying effect.