MEVALONATE KINASE IN LYSATES OF CULTURED HUMAN-FIBROBLASTS AND LYMPHOBLASTS - KINETIC-PROPERTIES, ASSAY CONDITIONS, CARRIER DETECTION AND MEASUREMENT OF RESIDUAL ACTIVITY IN A PATIENT WITH MEVALONIC ACIDURIA

被引:25
作者
GIBSON, KM
LOHR, JL
BROOCK, RL
HOFFMANN, G
NYHAN, WL
SWEETMAN, L
BRANDT, IK
WAPPNER, RS
BADER, PI
机构
[1] PARKVIEW MEM HOSP,FORT WAYNE,IN
[2] INDIANA UNIV,DEPT PEDIAT,INDIANAPOLIS,IN 46204
[3] INDIANA UNIV,DEPT MED GENET,INDIANAPOLIS,IN 46204
[4] JAMES WHITCOMB RILEY HOSP CHILDREN,INDIANAPOLIS,IN 46202
关键词
D O I
10.1159/000469050
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
引用
收藏
页码:47 / 55
页数:9
相关论文
共 16 条
  • [1] MEVALONIC ACIDURIA - AN INBORN ERROR OF CHOLESTEROL-BIOSYNTHESIS
    BERGER, R
    SMIT, GPA
    SCHIERBEEK, H
    BIJSTERVELD, K
    LECOULTRE, R
    [J]. CLINICA CHIMICA ACTA, 1985, 152 (1-2) : 219 - 222
  • [2] BEYTIA E, 1970, J BIOL CHEM, V245, P5450
  • [3] THE ANALYSIS OF ACYL-COENZYME A DERIVATIVES BY REVERSE-PHASE HIGH-PERFORMANCE LIQUID-CHROMATOGRAPHY
    DEBUYSERE, MS
    OLSON, MS
    [J]. ANALYTICAL BIOCHEMISTRY, 1983, 133 (02) : 373 - 379
  • [4] GARCIAMARTINEZ J, 1982, REV ESP FISIOL, V38, P393
  • [5] GARCIAMARTINEZ J, 1982, REV ESP FISIOL, V38, P261
  • [6] HARWOOD HJ, 1982, J LIPID RES, V23, P754
  • [7] MEVALONIC ACIDURIA - AN INBORN ERROR OF CHOLESTEROL AND NONSTEROL ISOPRENE BIOSYNTHESIS
    HOFFMAN, G
    GIBSON, KM
    BRANDT, IK
    BADER, PI
    WAPPNER, RS
    SWEETMAN, L
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1986, 314 (25) : 1610 - 1614
  • [8] AN IMPROVED PURIFICATION PROCEDURE, AN ALTERNATIVE ASSAY AND ACTIVATION OF MEVALONATE KINASE BY ATP
    LEE, CS
    OSULLIVAN, WJ
    [J]. BIOCHIMICA ET BIOPHYSICA ACTA, 1983, 747 (03) : 215 - 224
  • [9] PHOSPHOROTHIOATE ANALOGS OF ADENOSINE-TRIPHOSPHATE AS SUBSTRATES OF THE MEVALONATE KINASE REACTION
    LEE, CS
    OSULLIVAN, WJ
    [J]. BIOCHIMICA ET BIOPHYSICA ACTA, 1984, 787 (02) : 131 - 137
  • [10] LOWRY OH, 1951, J BIOL CHEM, V193, P265