DIRECT DEMONSTRATION THAT THE DEFICIENT OXIDATION OF VERY LONG-CHAIN FATTY-ACIDS IN X-LINKED ADRENOLEUKODYSTROPHY IS DUE TO AN IMPAIRED ABILITY OF PEROXISOMES TO ACTIVATE VERY LONG-CHAIN FATTY-ACIDS

被引：149

作者：

WANDERS, RJA

VANROERMUND, CWT

VANWIJLAND, MJA

SCHUTGENS, RBH

VANDENBOSCH, H

SCHRAM, AW

TAGER, JM

机构：

[1] STATE UNIV UTRECHT,BIOCHEM LAB,3584 CH UTRECHT,NETHERLANDS

[2] UNIV AMSTERDAM,BIOCHEM LAB,1000 HD AMSTERDAM,NETHERLANDS

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1988年 / 153卷 / 02期

关键词：

D O I：

10.1016/S0006-291X(88)81140-9

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：618 / 624

页数：7

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[21] X-LINKED ADRENOLEUKODYSTROPHY - DEFECTIVE PEROXISOMAL OXIDATION OF VERY LONG-CHAIN FATTY-ACIDS BUT NOT OF VERY LONG-CHAIN FATTY ACYL-COA ESTERS
WANDERS, RJA
VANROERMUND, CWT
VANWIJLAND, MJA
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SCHRAM, AW
TAGER, JM
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[22] STUDIES ON THE PEROXISOMAL OXIDATION OF PALMITATE AND LIGNOCERATE IN RAT-LIVER
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[23] PEROXISOMAL VERY LONG-CHAIN FATTY-ACID BETA-OXIDATION IN HUMAN-SKIN FIBROBLASTS - ACTIVITY IN ZELLWEGER SYNDROME AND OTHER PEROXISOMAL DISORDERS
WANDERS, RJA
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[24] ISOLATION OF RAT-LIVER LYSOSOMES BY ISOPYCNIC CENTRIFUGATION IN A METRIZAMIDE GRADIENT
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