GAL is a hydrolase in the lysosome, and its content is high in the renal proximal convoluted tubule epithelial cells.The activity of GAL in urine can reflect the renal parenchyma, especially the early damage of renal tubules. The determination of GAL activity in urine together with the determination of N-acetyl - β - D-glucosaminidase (NAG) in urine can be used for urine zymogram analysis, which is helpful for the observation of the course of disease and the evaluation of prognosis.In the field of genetics, the diagnosis (including prenatal diagnosis) and basic research of human β - galactosidase deficiency disease are also important indications.
The excretion rate of GAL in serum 0.2-0.4U/L urine, with reference range of 2.5-14.3IU/gCr.
Clinical significance
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Urine GAL and NAG are both lysosomal enzymes, mainly derived from renal tubular epithelial cells, so they have relatively sensitive responses to renal tubular injury and acquired diseases. At different stages of renal parenchyma injury, the excretion rate curves of GAL and NAG are somewhat different, and their use in enzyme spectrum analysis will help clinical diagnosis and prognosis evaluation.
matters needing attention
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(1) The linear range of this method can reach 220IU/L.(2) Buffer pH has a significant impact on extinction coefficient, and it is required to accurately correct the pH to the specified value.(3) The rest can be referred to "N-acetyl - β - D-glucosaminidase".(4) For application in genetics, please refer to relevant monographs and literatures.
Related diseases
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Angiokeratoma syndrome and ganglioside deposition disease in children
