Coproporphyrin I

From NuGOwiki

The NuGOwiki Metabolite Database is a joint initiative of NuGO and HMDB

Taxonomy Navigation Box; search by

Coproporphyrin I
2D structure for Coproporphyrin I
Chemical Name	3,8,13,18-tetramethyl-21H,23H-Porphine-2,7,12,17-tetrapropanoic acid
Chemical Formula	C₃₆H₃₈N₄O₈
CAS Number	531-14-6
Chemical Information	HMDB00643
Biochemical Taxonomy	Porphyrins
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Urine
Tissue Location	Liver Predominantly
Normal Biofluid Concentrations	Blood: 0.005 (0.0 - 0.01) uM Blood: 0.0073 +/- 0.0035 uM Blood: 0.012 +/- 0.005 uM Urine: 0.0036 +/- 0.0015 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Erythropoietic Protoporphyria Porphyria cutanea tarda
Other (Monogenic Disorders)	Dubin-johnson syndrome OMIM: 237500
Abnormal Biofluid Concentrations	Blood (Erythropoietic Protoporphyria): 0.085 (0.0008 - 0.17) uM Blood (Porphyria cutanea tarda): 0.008 (0.0 - 0.016) uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

Authors:
Affiliations:

Introduction

guidelines
Coproporhyrin I is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin I is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia. Porphyria is a pathological state characterised by abnormalities of porphyrin metabolism and results in the excretion of large quantities of porphyrins in the urine and in extreme sensitivity to light. A large number of factors are capable of increasing porphyrin excretion, owing to different and multiple causes and etiologies: 1) the main site of the chronic hepatic porphyria disease process concentrates on the liver, 2) a functional and morphologic liver injury is almost regularly associated with this chronic porphyria, 3) the toxic form due to occupational and environmental exposure takes mainly a subclinical course. Hepatic factors includes disturbance in coproporphyrinogen metabolism, which results from inhibition of coproporphyrinogen oxidase as well as from the rapid loss from, and diminished utilization of coproporphyrinogen in the hepatocytes, which may also explain why coproporphyrin, its autoxidation product, predominates physiologically in the urine; decreased biliary excretion of coproporphyrin leading to a compensatory urinary excretion, so that the coproporphyrin ring isomer ratio (1:III) becomes a sensitive index for impaired liver function and intrahepatic cholestasis; and disturbed activity of hepatic uroporphyrinogen decarboxylase. In itself, secondary coproporphyrinuria is not associated with porphyria symptoms of a hepatologic-gastroenterologic, neurologic, or dermatologic order, even though coproporphyrinuria can occur with such symptoms. (PMID: 3327428)