5-aminolevulinic acid (ALA) is an early precursor in the synthesis of the heme, which is involved in the composition of haemoglobin, and is synthesised in the mitochondrion.
An enzyme deficiency in the porphyrin pathway (5- aminolevulinate dehydrogenase) leads to insufficient production of heme and accumulation of ALA. This anomaly is encountered in hepatic porphyria and is one of the types of porphyrias, inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway.
After sample preparation the sample is measured on our UVIKON XS double beam UV/Vis spectrophotometer at 553nm. The results are normalised to creatinine concentration and reported as a ratio of amounts (micromoles of ALA / millimoles of creatinine).
- Up to 20 samples per batch measured either sequentially or using an automatic cell changer
- Sample number and or patient name can be entered
- 553nm absorbance and ALA calculation (umol/l) automatically performed
- Selectable ALA upper and lower concentration warning limits
- Sample dilution taken into consideration for calculation
- ALA / Creatinine ratio calculated (umol/mmol)