STUDIES ON THE URINARY-EXCRETION OF THROMBOXANE B-2 IN ZELLWEGER PATIENTS AND CONTROL SUBJECTS - EVIDENCE FOR A MAJOR ROLE FOR PEROXISOMES IN THE BETA-OXIDATIVE CHAIN-SHORTENING OF THROMBOXANE B-2

被引:9
作者
DEWAART, DR [1 ]
KOOMEN, GCM [1 ]
WANDERS, RJA [1 ]
机构
[1] UNIV AMSTERDAM,ACAD MED CTR,DEPT PEDIAT,1105 AZ AMSTERDAM,NETHERLANDS
来源
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE | 1994年 / 1226卷 / 01期
关键词
PEROXISOME; BETA-OXIDATION; THROMBOXANE; ZELLWEGER SYNDROME; EICOSANOID;
D O I
10.1016/0925-4439(94)90057-4
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
In this paper we studied the urinary excretion of thromboxane B-2 and its beta-oxidation product 2,3-dinor-thromboxane B-2 in urines from control subjects and four Zellweger patients, which lack morphologically distinguishable peroxisomes. In the urine of three classical Zellweger patients we found a ratio of 2,3-dinor-thromboxane B-2/thromboxane B-2 of 0.35, 0.48 and 0.62 respectively, whereas in healthy children and adults values were found of 3.1-10 and 5.5-40 respectively. These data strongly suggest that peroxisomes are a major site for beta-oxidation of thromboxane B-2.
引用
收藏
页码:44 / 48
页数:5
相关论文
共 24 条
[1]   A SIBSHIP WITH A MILD VARIANT OF ZELLWEGER SYNDROME [J].
BARTH, PG ;
SCHUTGENS, RBH ;
WANDERS, RJA ;
HEYMANS, HSA ;
MOSER, AE ;
MOSER, HW ;
BLEEKERWAGEMAKERS, EM ;
JANSONIUSSCHULTHEISS, K ;
DERIX, M ;
NELCK, GF .
JOURNAL OF INHERITED METABOLIC DISEASE, 1987, 10 (03) :253-259
[2]  
BREMER J, 1982, J LIPID RES, V23, P243
[3]   EFFECT OF CLOFIBRATE-FEEDING ON HEPATIC FATTY-ACID METABOLISM [J].
CHRISTIANSEN, RZ .
BIOCHIMICA ET BIOPHYSICA ACTA, 1978, 530 (03) :314-324
[4]  
DICZFALUSY U, 1988, J LIPID RES, V29, P1629
[5]   CHAIN-SHORTENING OF PROSTAGLANDIN-F2-ALPHA BY RAT-LIVER PEROXISOMES [J].
DICZFALUSY, U ;
ALEXSON, SEH ;
PEDERSEN, JI .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1987, 144 (03) :1206-1213
[6]   CORONARY ARTERIAL SMOOTH-MUSCLE CONTRACTION BY A SUBSTANCE RELEASED FROM PLATELETS - EVIDENCE THAT IT IS THROMBOXANE-A2 [J].
ELLIS, EF ;
OELZ, O ;
ROBERTS, LJ ;
PAYNE, NA ;
SWEETMAN, BJ ;
NIES, AS ;
OATES, JA .
SCIENCE, 1976, 193 (4258) :1135-1137
[7]   THROMBOXANES - NEW GROUP OF BIOLOGICALLY-ACTIVE COMPOUNDS DERIVED FROM PROSTAGLANDIN ENDOPEROXIDES [J].
HAMBERG, M ;
SVENSSON, J ;
SAMUELSSON, B .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1975, 72 (08) :2994-2998
[8]  
HILTUNEN JK, 1986, J BIOL CHEM, V261, P6484
[9]   PEROXISOMAL BETA-OXIDATION OF LONG-CHAIN FATTY-ACIDS POSSESSING DIFFERENT EXTENTS OF UNSATURATION [J].
HOVIK, R ;
OSMUNDSEN, H .
BIOCHEMICAL JOURNAL, 1987, 247 (03) :531-535
[10]  
JEDLITSCHKY G, 1991, J BIOL CHEM, V266, P24763