Mauve Factor, Kryptopyrrole and Pyrroluria
“Mauve Factor,” or “Mauve”, was first detected in the urine of psychiatric patients in 1958 .
Mauve was identified mistakenly as kryptopyrrole (KP) in a high-profile scientific journal in 1969 . Improved technology, however, demonstrated that KP is not found in human urine [3, 4], and Mauve was identified indisputably as hydroxyhemopyrrolin-2-one, or HPL [5, 6].
Therefore, “High-Mauve” denotes subjects or groups with elevated HPL or with a tendency to excrete excess HPL, and should be used instead of the term “pyrroluria” which lacks specificity, as many pyrroles appear in urine .
What is HPL?
The exact biological origin of HPL (Mauve Factor) is unknown .
HPL may be a product of isocoproporphyrins, abnormal porphyrins (pigments) that result from altered heme production . Hemes are most commonly recognized as components of hemoglobin, the red pigment in blood, but are also found in a number of other biologically important hemoproteins such as myoglobin, cytochrome, catalase, heme peroxidase, and endothelial nitric oxide synthase.
The formation of isocoproporphyrin requires the participation of gut bacteria. Evidence does suggest bacterial involvement in the formation of Mauve, as antibiotics reversibly abolish urinary HPL excretion .
All humans seem to excrete small quantities of HPL in urine .
The normal concentration of HPL in urine is estimated at 2 to 25 μg/dL .
Urinary HPL levels over twice the upper limit of normal are considered highly elevated, but very high HPL measurements – hundreds of micrograms per deciliter – are also reported .
HPL is also detectable in human blood and cerebrospinal fluid .
Why is too much of HPL Bad?
High-Mauve was reported in psychosis, alcoholism and substance abuse, psychoneurosis, and in many cognitive, effective, and neurobehavioral disorders .
Several findings suggest that HPL is neurotoxic in humans. As a class, pyrroles have been called “nerve poisons”, and HPL is from the subclass of monopyrroles, some of which cause acute neurobehavioral effects in animals .
Injection of HPL decreased activity in rats, while increasing aggressive behavior, while a higher dose of HPL increased head-twitch and backward locomotion, behaviors usually associated with hallucinogens .
Individuals with high-Mauve cannot efficiently create serotonin (a neurotransmitter that contributes to feelings of well-being and happiness and reduces anxiety and depression) since vitamin B6 is an important factor in the last step of serotonin production .
Heme depression further results in excess nitric oxide (NO) , which is injurious to the brain [16, 17] and is suspected to play a role in such high-Mauve disorders as schizophrenia and autism [18, 19]. Plasma NO and urinary HPL were positively associated .
Table 1. Neurobehavioral disorders associated with elevated HPL .
What are the Symptoms of High Mauve?
- poor stress control
- nervousness, anxiety
- mood swings
- severe inner tension
- episodic anger (explosive temper)
- poor short-term memory
- poor dream recall
- inability to tan
- sensitivity to light and sound
- morning nausea
- very dry skin
- white spots on the fingernails
- stretch marks on the skin
- poor growth
- spleen area pain
- joint pain
- abnormal or absent menstrual periods
Poor dream recall and mild morning nausea/breakfast anorexia may relate especially to B6 deficiency. White flecks in the nails are responsive to zinc, while stretch marks result from a combined deficiency of B6 and zinc .
Table 2. Symptoms prevalent in high-Mauve patients .
Neurobehavioral symptoms may improve after only a few days of therapy with B6 and zinc, but discontinuation may result in severe deterioration within 48 hours .
Vitamin B6 (200-800 mg daily) in combination with zinc (25-100 mg daily) usually is sufficient to suppress HPL and achieve optimal symptomatic response .
Long-term treatment with B6 and zinc is needed for ongoing HPL suppression and symptom management. Optimal initial dosages may be higher than maintenance dosages. Zinc requirements in high-Mauve subjects are noted to increase during growth spurts then decline abruptly .
Generally, higher urinary HPL suggests the need for proportionately higher dosing of zinc and B6, and repeated measurements of HPL should influence dosing decisions. Clinical symptoms and HPL suppression are the primary determinants of B6 dosing .
P5P may be effective in combination with B6 or instead of B6 in some high-Mauve subjects .
Long-term treatment of high-Mauve patients with high dose vitamin B6 resulted in no cases of peripheral neuropathy, but reversible median nerve paresthesis (numbness and tingling) has been reported .
In adults receiving supplements, cellular or plasma zinc should not exceed upper limits of normal, and cellular or serum copper should not be below the normal range .
Suppression of manganese may result from aggressive zinc supplementation. Small dosages (approximately 5 mg manganese for each 30 mg of supplemental zinc) reportedly improve symptoms in some high-Mauve subjects. However, serum or red-cell levels should be monitored during supplementation with manganese, which in excess is pro-oxidant .
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