Systematic errors in initial substrate concentration (s(0)), product concentration and reaction time give much larger errors in the Michaelis-Menten parameters unless s(0) is treated as an unknown parameter. These errors are difficult to detect because the fitted curve deviates little from the data. The effect of non-enzymic reaction is also examined.
The case history, laboratory findings and properties of the enzyme in a patient with hexokinase deficiency are reported. The mother had a haemolytic disorder of similar severity. In these cases the expression of the disease in both mother and son suggests a dominant mode of inheritance. This family further illustrates the necessity of taking the reticulocyte level into account when interpreting red cell enzyme levels.
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