Porphyrin and heme metabolism and the porphyrias

HL Bonkovsky, JT Guo, W Hou, T Li… - Comprehensive …, 2013 - Wiley Online Library
Porphyrins and metalloporphyrins are the key pigments of life on earth as we know it,
because they include chlorophyll (a magnesium‐containing metalloporphyrin) and heme …

Porphyrias: a 2015 update

Z Karim, S Lyoumi, G Nicolas, JC Deybach… - Clinics and research in …, 2015 - Elsevier
The hereditary porphyrias comprise a group of eight metabolic disorders of the heme
biosynthesis pathway. Each porphyria is caused by abnormal function at a separate …

Clinically important features of porphyrin and heme metabolism and the porphyrias

S Besur, W Hou, P Schmeltzer, HL Bonkovsky - Metabolites, 2014 - mdpi.com
Heme, like chlorophyll, is a primordial molecule and is one of the fundamental pigments of
life. Disorders of normal heme synthesis may cause human diseases, including certain …

[HTML][HTML] Acute hepatic porphyria

DM Bissell, B Wang - Journal of clinical and translational …, 2015 - ncbi.nlm.nih.gov
The porphyrias comprise a set of diseases, each representing an individual defect in one of
the eight enzymes mediating the pathway of heme synthesis. The diseases are genetically …

The acute hepatic porphyrias: current status and future challenges

M Siegesmund, AMT van Serooskerken… - Best Practice & …, 2010 - Elsevier
The porphyrias are predominantly inherited metabolic disorders, which result from a specific
deficiency of one of the eight enzymes along the pathway of haem biosynthesis. Historically …

[PDF][PDF] Acute hepatic porphyrias: review and recent progress

B Wang, S Rudnick, B Cengia… - Hepatology …, 2019 - Wiley Online Library
The acute hepatic porphyrias (AHPs) are a group of four inherited diseases of heme
biosynthesis that present with episodic, acute neurovisceral symptoms. The four types are 5 …

Porphyrins, porphyrin metabolism and porphyrias. I. Update

S Thunell - Scandinavian journal of clinical and laboratory …, 2000 - Taylor & Francis
The biosynthesis of porphyrins is one of the most conserved parthways known, about the
same sequence of reactions taking place in all species. By associating different metals …

Pathogenesis and clinical features of the acute hepatic porphyrias (AHPs)

HL Bonkovsky, N Dixon, S Rudnick - Molecular genetics and metabolism, 2019 - Elsevier
The acute hepatic porphyrias include four disorders: acute intermittent porphyria [AIP],
hereditary coproporphyria [HCP], variegate porphyria [VP], and the rare porphyria due to …

Identification of the mitochondrial heme metabolism complex

AE Medlock, MT Shiferaw, JR Marcero, AA Vashisht… - PloS one, 2015 - journals.plos.org
Heme is an essential cofactor for most organisms and all metazoans. While the individual
enzymes involved in synthesis and utilization of heme are fairly well known, less is known …

Terminal steps of haem biosynthesis

HA Dailey - Biochemical Society Transactions, 2002 - portlandpress.com
The terminal three steps in haem biosynthesis are the oxidative decarboxylation of
coproporphyrinogen III to protoporphyrinogen IX, followed by the six-electron oxidation of …