Liver metabolism of porphyrins and haem

@article{Bloomer1998LiverMO,
  title={Liver metabolism of porphyrins and haem},
  author={Joseph R. Bloomer},
  journal={Journal of Gastroenterology and Hepatology},
  year={1998},
  volume={13}
}
  • J. Bloomer
  • Published 1 March 1998
  • Medicine, Computer Science, Biology
  • Journal of Gastroenterology and Hepatology
The liver is an active site for the biosynthesis of haem and porphyrinogens/porphyrins, which are intermediates of the haem biosynthetic pathway, because haem is required for functional activity of the cytochrome P 450 system and other critical hepatic haemoproteins. The production of hepatic haem is regulated primarily through the activity of aminolaevulinic acid synthase which is the first and normally rate‐limiting enzyme of the pathway. This is, in turn, controlled by a putative regulatory… 
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References

SHOWING 1-10 OF 43 REFERENCES

EFFECT OF HAEM ARGINATE THERAPY ON PORPHYRIN METABOLISM AND MIXED FUNCTION OXYGENASE ACTIVITY IN ACUTE HEPATIC PORPHYRIA

Effects of haem infusion on biliary secretion of porphyrins, haem and bilirubin in man

TLDR
A few patients with representative porphyrias showed the expected increase in copro‐ and protoporphyrin in the bile, including the patient with protoporphyria, which exhibited a bile flow of coproporphyrine of 1470 pL 133 nmol/h and of protopomorphyrin of 334 pL 29 nmol /h.

Intravenous heme-albumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools.

TLDR
Infusions of heme administered intravenously, complexed to human serum albumin are safe and effective in repleting deficient heme pools and hemoproteins in patients with acute porphyria and that activities of cytochrome(s) P-450 in normal subjects may also be increased by heme administration.

Hepatic heme metabolism and cytochrome P450 in cirrhotic rat liver.

Relationship between biliary lipid and protoporphyrin secretion; potential role of mdr2 P-glycoprotein in hepatobiliary organic anion transport.

Hepatic and bile porphyrins in patients with protoporphyria and liver failure.

Haem arginate improves hepatic oxidative metabolism in variegate porphyria.

TLDR
The severe impairment of hepatic mixed function oxidase activity even in the symptomless stage of porphyria indicates cautious dosage of drugs primarily eliminated by hepatic oxidative reactions.

Rapid normalization of antipyrine oxidation by heme in variegate porphyria

TLDR
It is concluded that in variegate porphyria a partial block in heme biosynthesis results in subnormal capacity of P450‐associated monooxygenases, but this is easily normalized by exogenous heme.

Secretion of haem by hepatic parenchymal cells.

TLDR
In cultures incubated with the haem precursor delta-amino[4-14C]laevulinate, labelled haem was formed at a linear rate for at least 8 h, and 10-20% of the total labelled Haem was present in the culture medium.

Degradation of endogenous hepatic heme by pathways not yielding carbon monoxide. Studies in normal rat liver and in primary hepatocyte culture.

TLDR
It is concluded that heme associated with microsomal cytochromes in normal rat liver is degraded substantially by non-CO forming processes.