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Copper coproporphyrin excretion in familial coproporphyria
Clinical Chemistry
|November 1, 1978
Summary
Familial coproporphyria patients excrete significant amounts of copper coproporphyrin in their stool. This copper coproporphyrin likely forms nonenzymically from coproporphyrin and copper in the bile or feces.
Area of Science:
- Biochemistry
- Human Genetics
- Analytical Chemistry
Background:
- Familial coproporphyria is a rare genetic disorder.
- Porphyrias are a group of inherited or acquired disorders of heme biosynthesis.
Purpose of the Study:
- To analyze the porphyrin profile in stool specimens from patients with familial coproporphyria.
- To identify and quantify specific porphyrin compounds, particularly copper coproporphyrin.
Main Methods:
- High-performance liquid chromatography (HPLC) was used for the quantitative analysis of porphyrins in stool.
- Stool specimens from familial coproporphyria patients were examined.
Main Results:
- Copper coproporphyrin was identified as a significant component (14% of total porphyrin) in the stool of a familial coproporphyria patient.
- Other patients with this disease also showed substantial levels of both copper coproporphyrin and coproporphyrin.
- Evidence suggests copper coproporphyrin is formed through nonenzymic copper incorporation into coproporphyrin within the bile or feces.
Conclusions:
- Copper coproporphyrin is a notable fecal porphyrin in familial coproporphyria.
- The nonenzymic formation of copper coproporphyrin in the gut or bile is a likely mechanism.
- Further research into the implications of copper coproporphyrin in this condition is warranted.