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Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in urine by use of the Bratton-Marshall reaction: identification of patients deficient in adenylosuccinate lyase activity.
Anal Biochem 1986; 156(1):81-90AB

Abstract

The Bratton-Marshall reaction can be used to identify patients with adenylosuccinate lyase deficiency. These patients excrete in their urine the dephosphorylated derivative of the de novo purine synthesis intermediate 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole (SAICAR). The test described here depends on a coupling reaction of N-1-naphthylethylenediamine with diazotized ribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole giving rise to a fast developing purple chromaphore with a maximum absorbance at 555 nm. Using the closely related compound ribosyl-5-amino-4-imidazolecarboxamide (AICA riboside) as a standard, concentrations as low as 1.0 microM produce a visible color change. The absorption at 555 nM of the azo compound increases as a linear function of the concentration of AICA riboside in the reaction. The use of a filter-paper dipstick for urine sampling and storage is also described. The two metabolites which are present in increased concentration in biological fluids of adenylosuccinate lyase deficient patients are stable on the dipstick for at least 60 days when stored at room temperature (25 degrees C).

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

3740420

Citation

Laikind, P K., et al. "Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in Urine By Use of the Bratton-Marshall Reaction: Identification of Patients Deficient in Adenylosuccinate Lyase Activity." Analytical Biochemistry, vol. 156, no. 1, 1986, pp. 81-90.
Laikind PK, Seegmiller JE, Gruber HE. Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in urine by use of the Bratton-Marshall reaction: identification of patients deficient in adenylosuccinate lyase activity. Anal Biochem. 1986;156(1):81-90.
Laikind, P. K., Seegmiller, J. E., & Gruber, H. E. (1986). Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in urine by use of the Bratton-Marshall reaction: identification of patients deficient in adenylosuccinate lyase activity. Analytical Biochemistry, 156(1), pp. 81-90.
Laikind PK, Seegmiller JE, Gruber HE. Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in Urine By Use of the Bratton-Marshall Reaction: Identification of Patients Deficient in Adenylosuccinate Lyase Activity. Anal Biochem. 1986;156(1):81-90. PubMed PMID: 3740420.
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TY - JOUR T1 - Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in urine by use of the Bratton-Marshall reaction: identification of patients deficient in adenylosuccinate lyase activity. AU - Laikind,P K, AU - Seegmiller,J E, AU - Gruber,H E, PY - 1986/7/1/pubmed PY - 1986/7/1/medline PY - 1986/7/1/entrez SP - 81 EP - 90 JF - Analytical biochemistry JO - Anal. Biochem. VL - 156 IS - 1 N2 - The Bratton-Marshall reaction can be used to identify patients with adenylosuccinate lyase deficiency. These patients excrete in their urine the dephosphorylated derivative of the de novo purine synthesis intermediate 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole (SAICAR). The test described here depends on a coupling reaction of N-1-naphthylethylenediamine with diazotized ribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole giving rise to a fast developing purple chromaphore with a maximum absorbance at 555 nm. Using the closely related compound ribosyl-5-amino-4-imidazolecarboxamide (AICA riboside) as a standard, concentrations as low as 1.0 microM produce a visible color change. The absorption at 555 nM of the azo compound increases as a linear function of the concentration of AICA riboside in the reaction. The use of a filter-paper dipstick for urine sampling and storage is also described. The two metabolites which are present in increased concentration in biological fluids of adenylosuccinate lyase deficient patients are stable on the dipstick for at least 60 days when stored at room temperature (25 degrees C). SN - 0003-2697 UR - https://www.unboundmedicine.com/medline/citation/3740420/Detection_of_5'_phosphoribosyl_4__N_succinylcarboxamide__5_aminoimidazole_in_urine_by_use_of_the_Bratton_Marshall_reaction:_identification_of_patients_deficient_in_adenylosuccinate_lyase_activity_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0003-2697(86)90158-2 DB - PRIME DP - Unbound Medicine ER -