A sensitive, accurate method has been established for the assay of serum carnosinase by measuring the fluorescence emitted from the L-histidine liberated on treatment with o-phthaldialdehyde. Using this method the serum values for normal adults, infants and children were measured. The mean value was very low in infants of less than 1 year old but increased with age, being almost the same in children aged 6 years or more as in adults. In adult men, the mean activity was 1·85 μmol/mL/h and in adult women it was 2·07 μmol/mL/h. Low activity was observed in patients with muscular dystrophy.
References
1.
EmilLS. Dipeptidase: a carnosinase. In: Colo-WickSPKaplanNO, eds. Methods in Enzymology. Vol. 2. New York: Academic Press, 1969; 93–6.
2.
MurpheyWHPatchenLLindmarkDG. Carnosinase: A fluorometric assay and demonstration of two electrophoretic forms in human tissue extracts. Clin Chim Acta1972; 42: 309–14.
3.
GerberDA. Determination of histidine in serum with o-phthaldialdehyde. Anal BioChem.1970; 34: 500–4.
4.
ShorePABurkhalterAChonVH. A method for the fluorometric assay of histidine. J Pharmacol Exp Ther1959; 127: 182–6.
5.
PerryTLHansenSTischlerB, Carnosinemia: A new metabolic disorder associated with neurologic disease and mental defect. N Engl J Med.1967; 277: 1219–27.
6.
HartlagePLRoeselRAHommesFA. Serum carnosinase deficiency: decreased affinity of the enzyme for the substrate. J Inherit Metab Dis.1982; 5 Suppl.1: 13–4.
PerryTLHansenSLoveDL. Serum-carnosinase deficiency in carnosinemia. Lancet.1958; 1: 1229–34.
9.
BurgessEAOberholzerVGPalmerT, Plasma carnosinase deficiency in patients with urea cycle defects. Clin Chim Acta1975; 61: 215–8.
10.
RoeselRAHartlagePLHallWK. Carnosinuria and serum carnosinase deficiency in a child with congenital myopathy. Fed Proc1981; 40: 461–5.
11.
Van MunsterPJTreibelsJMFMoerkerkC. A new sensitive method for the determination of serum carnosinase activity using 1-carnosine-(1C14) β-alanyl as substrate. Clin Chim Acta1970; 29: 243–8.
12.
JamesFLRobertPGAnnaMW, Human serum carnosinase: characterization, distinction from cellular carnosinase and activation by cadmium. Clin Chim Acta1982; 123: 221–31.