HUMAN LIVER L-ALANINE GLYOXYLATE AMINOTRANSFERASE - CHARACTERISTICS AND ACTIVITY IN CONTROLS AND HYPEROXALURIA TYPE-I PATIENTS USING A SIMPLE SPECTROPHOTOMETRIC METHOD

被引:22
作者
WANDERS, RJA
RUITER, J
VANROERMUND, CWT
SCHUTGENS, RBH
OFMAN, R
JURRIAANS, S
TAGER, JM
机构
[1] UNIV AMSTERDAM HOSP,DEPT PEDIAT,AMSTERDAM,NETHERLANDS
[2] UNIV AMSTERDAM HOSP,DEPT CLIN CHEM,AMSTERDAM,NETHERLANDS
[3] UNIV AMSTERDAM,BIOCHEM LAB,AMSTERDAM,NETHERLANDS
关键词
Alanine-glyoxylate aminotransferase; Oxalate; Peroxisomal disorder; Peroxisome; Primary hyperoxaluria type I;
D O I
10.1016/0009-8981(90)90084-6
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
We have studied the characteristics of human liver alanine-glyoxylate aminotransferase, which is deficient in hyperoxaluria type I, an inherited disorder of glyoxylate metabolism. The enzyme was optimally active at pH 8.0 showing apparent Km values for l-alanine and glyoxylate of 8.3 and 1.3 mmol/l, respectively. Activity was found to proceed linearly for up to 4 h. Measurements under these optimal conditions enabled the biochemical diagnosis of hyperoxaluria type I to be made via enzyme activity measurements in percutaneous needle biopsy specimens of liver tissue. © 1990.
引用
收藏
页码:139 / 144
页数:6
相关论文
共 9 条
[1]   A NEW MICRO-ASSAY FOR HUMAN-LIVER ALANINE - GLYOXYLATE AMINOTRANSFERASE [J].
ALLSOP, J ;
JENNINGS, PR ;
DANPURE, CJ .
CLINICA CHIMICA ACTA, 1987, 170 (2-3) :187-193
[2]   PEROXISOMAL ALANINE - GLYOXYLATE AMINOTRANSFERASE DEFICIENCY IN PRIMARY HYPEROXALURIA TYPE-I [J].
DANPURE, CJ ;
JENNINGS, PR .
FEBS LETTERS, 1986, 201 (01) :20-24
[3]   PEROXISOMAL LOCALIZATION OF ALANINE - GLYOXYLATE AMINOTRANSFERASE IN HUMAN-LIVER [J].
NOGUCHI, T ;
TAKADA, Y .
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1979, 196 (02) :645-647
[4]   ASSAYS FOR GLYOXYLATE AMINOTRANSFERASE ACTIVITIES [J].
ROWSELL, EV ;
SNELL, K ;
TAKTAK, B ;
CARNIE, JA .
INTERNATIONAL JOURNAL OF BIOCHEMISTRY, 1972, 3 (15) :247-&
[5]  
THOMPSON JS, 1967, J BIOL CHEM, V242, P3614
[6]  
THOMPSON JS, 1986, ANAL BIOCHEM, V24, P197
[7]   ALANINE GLYOXYLATE AMINOTRANSFERASE AND THE URINARY-EXCRETION OF OXALATE AND GLYCOLATE IN HYPEROXALURIA TYPE-I AND THE ZELLWEGER SYNDROME [J].
WANDERS, RJA ;
VANROERMUND, CWT ;
WESTRA, R ;
SCHUTGENS, RBH ;
VANDERENDE, MA ;
TAGER, JM ;
MONNENS, LAH ;
BAADENHUYSEN, H ;
GOVAERTS, L ;
PRZYREMBEL, H ;
WOLFF, ED ;
BLOM, W ;
HUIJMANS, JGM ;
VANLAERHOVEN, FGM .
CLINICA CHIMICA ACTA, 1987, 165 (2-3) :311-319
[8]   DIVERSITY IN RESIDUAL ALANINE GLYOXYLATE AMINOTRANSFERASE ACTIVITY IN HYPEROXALURIA TYPE-I - CORRELATION WITH PYRIDOXINE RESPONSIVENESS [J].
WANDERS, RJA ;
VANROERMUND, CWT ;
JURRIAANS, S ;
SCHUTGENS, RBH ;
TAGER, JM ;
VANDENBOSCH, H ;
WOLFF, ED ;
PRZYREMBEL, H ;
BERGER, R ;
SCHAAPHOK, FG ;
REITSMA, W ;
VANLUYK, WHJ .
JOURNAL OF INHERITED METABOLIC DISEASE, 1988, 11 :208-211
[9]  
WILLIAMS HE, 1983, METABOLIC BASIS INHE, P204