Pyroluria

    Pyroluria is a genetic blood disorder caused by a chemical imbalance involving an abnormality in the synthesis of hemoglobin. During the production of hemoglobin, there is a seemingly harmless byproduct called pyrrole, where the increased excretion of this product is measured by hydroxyhemopyrroline-2-one (HPL):

[HPL] Below 10 μg/dl: Normal

[HPL] Between 10-20 μg/dl: Borderline (considered high if there is a clinical correlation)

[HPL] Above 20 μg/dl: High

In pyroluria, pyrrole levels are high, and systematically bind to and block the nutrients vitamin B6 and zinc. Thus, biochemical pathways that require these nutrients as cofactors are thus impaired:

⮚ synthesis and regulation of GABA requires zinc and vitamin B6;

⮚ Vitamin B6 (P5P) is a cofactor in the conversion of L-Dopa to dopamine;

⮚ Serotonin synthesis from 5HTP requires B6 (P5P) and zinc;

⮚ Oxidative deamination by DAO requires B6 (PLP) as a cofactor.