PEROXISOMAL FATTY-ACID BETA-OXIDATION IN RELATION TO THE ACCUMULATION OF VERY LONG-CHAIN FATTY-ACIDS IN CULTURED SKIN FIBROBLASTS FROM PATIENTS WITH ZELLWEGER SYNDROME AND OTHER PEROXISOMAL DISORDERS

被引：80

作者：

WANDERS, RJA

VANROERMUND, CWT

VANWIJLAND, MJA

SCHUTGENS, RBH

HEIKOOP, J

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：

JOURNAL OF CLINICAL INVESTIGATION | 1987年 / 80卷 / 06期

关键词：

D O I：

10.1172/JCI113271

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

引用

页码：1778 / 1783

页数：6

共 51 条

[1] CHARACTERIZATION OF RAT-BRAIN MICROSOMAL ACYL-COENZYME-A LIGASES - DIFFERENT ENZYMES FOR THE SYNTHESIS OF PALMITOYL-COENZYME-A AND LIGNOCEROYL-COENZYME-A [J].

BHUSHAN, A ;

SINGH, RP ;

SINGH, I .

ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1986, 246 (01) :374-380

[2]

BJORKHEM I, 1985, STEROLS BILE ACIDS, P231

[3]

BREMER J, 1982, J LIPID RES, V23, P243

[4]

BREMER J, 1984, FATTY ACID METABOLIS, P113

[5] DEFICIENCY OF ENZYMES CATALYZING THE BIOSYNTHESIS OF GLYCEROL ETHER LIPIDS IN ZELLWEGER SYNDROME - A NEW CATEGORY OF METABOLIC DISEASE INVOLVING THE ABSENCE OF PEROXISOMES [J].

DATTA, NS ;

WILSON, GN ;

HAJRA, AK .

NEW ENGLAND JOURNAL OF MEDICINE, 1984, 311 (17) :1080-1083

[6]

DEGROOT PHE, 1976, ADV LIPID RES, P75

[7] TISSUE FRACTIONATION STUDIES .6. INTRACELLULAR DISTRIBUTION PATTERNS OF ENZYMES IN RAT-LIVER TISSUE [J].

DUVE, CD ;

PRESSMAN, BC ;

GIANETTO, R ;

WATTIAUX, R ;

APPELMANS, F .

BIOCHEMICAL JOURNAL, 1955, 60 (1-4) :604-617

[8] PEROXISOMAL DEFECTS IN NEONATAL-ONSET AND X-LINKED ADRENOLEUKODYSTROPHIES [J].

GOLDFISCHER, S ;

COLLINS, J ;

RAPIN, I ;

COLTOFFSCHILLER, B ;

CHANG, CH ;

NIGRO, M ;

BLACK, VH ;

JAVITT, NB ;

MOSER, HW ;

LAZAROW, PB .

SCIENCE, 1985, 227 (4682) :67-70

[9]

GOLDFISCHER S, 1984, INT REV EXP PATHOL, V26, P45

[10]

HAJRA AK, 1984, ETHER PHOSPHOLIPIDS, P85

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