Coproporphyrin III
Coproporphyrin III is a porphyrin metabolite arising from heme synthesis. Porphyrins are pigments found in both animal and plant life. Coproporphyrin III 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 reslits 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 mlitiple 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 regliarly 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 reslits 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 ).
Structure for HMDB00570 (Coproporphyrin III)
C36H38N4O8
654.7089
654.268964212
3-[10,14,19-tris(2-carboxyethyl)-5,9,15,20-tetramethyl-21,22,23,24-tetraazapentacyclo[16.2.1.1³,⁶.1⁸,¹¹.1¹³,¹⁶]tetracosa-1(21),2,4,6,8(23),9,11,13,15,17,19-undecaen-4-yl]propanoic acid
coproporphyrin III
14643-66-4
JWFCYWSMNRLXLX-UJJXFSCMSA-N
Not classified
Detected and Quantified
Not Available
Not Available
Solid
Predicted LC-MS/MS Spectrum – 10V, Positivesplash10-00kr-0000059000-ee622066b0f73e50cf91View in MoNA
Predicted LC-MS/MS Spectrum – 20V, Positivesplash10-00kp-0000094000-ee6c72d818b957fec0b6View in MoNA
Predicted LC-MS/MS Spectrum – 40V, Positivesplash10-01ot-0000090000-87edb6484e1fd9c98561View in MoNA
Predicted LC-MS/MS Spectrum – 10V, Negativesplash10-0f79-0000029000-08747adb62bb52441aebView in MoNA
Predicted LC-MS/MS Spectrum – 20V, Negativesplash10-052u-1000079000-d36d808ce647034f9109View in MoNA
Predicted LC-MS/MS Spectrum – 40V, Negativesplash10-052f-7000094000-b86bf714ce0924edc701View in MoNA
Porphyria
Porphyria
- Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
- Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed:10905759 ]
- Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19. [PubMed:10638943 ]
DB04461
Not Available
Not Available
Not Available
FDB005557
Not Available
16736509
C05770
Not Available
Not Available
Not Available
HMDB00570
HMDB00570
5553
Not Available
1R3T
27609
- Doss MO: Porphyrinurias and occupational disease. Ann N Y Acad Sci. 1987;514:204-18. [PubMed:3327428 ]
Enzymes
- General function:
- Involved in uroporphyrinogen decarboxylase activity
- Specific function:
- Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
- Gene Name:
- UROD
- Uniprot ID:
- P06132
- Molecular weight:
- 40786.58
References
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
- General function:
- Involved in coproporphyrinogen oxidase activity
- Specific function:
- Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III.
- Gene Name:
- CPOX
- Uniprot ID:
- P36551
- Molecular weight:
- 50151.605