AI Article Synopsis
- A spectrophotometric method for testing porphobilinogen deaminase in red blood cells is detailed, crucial for diagnosing acute intermittent porphyria.
- The test uses delta-aminolevulinic acid as a substrate, with mercaptoethanol and zinc to optimize conditions for producing porphobilinogen.
- Uroporphyrins are produced at 45 degrees Celsius and measured at 405 nm, allowing for easy implementation in clinical labs to identify patients and carriers of the condition.
Article Abstract
A spectrophotometric method for porphobilinogen deaminase assay in erythrocytes is described. This test is determinant for the definite diagnosis of acute intermittent porphyria. In the method described, delta-aminolevulinic acid is used as substrate. Mercaptoethanol and zinc ions are introduced to maintain delta-aminolevulinic acid dehydratase in optimal conditions and to guarantee the in vitro production of porphobilinogen. An incubation temperature of 45 degrees C leads to the production of uroporphyrins, which are measured spectrophotometrically at 405 nm, giving reproducible results. The assay can be performed easily in any clinical laboratory and is valuable for detecting both patients and carriers of acute intermittent porphyria.
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| http://dx.doi.org/10.1007/BF00711375 | DOI Listing |
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