BRAIN, LIVER, AND ADIPOSE-TISSUE ERUCIC AND VERY LONG-CHAIN FATTY-ACID LEVELS IN ADRENOLEUKODYSTROPHY PATIENTS TREATED WITH GLYCERYL TRIERUCATE AND TRIOLEATE OILS (LORENZO-OIL)

被引:55
作者
RASMUSSEN, M
MOSER, AB
BOREL, J
KHANGOORA, S
MOSER, HW
机构
[1] Department of Neurogenetics, The Kennedy Krieger Institute, Baltimore, 21205, MD
关键词
ADRENOLEUKODYSTROPHY; ERUCIC ACID; VERY LONG CHAIN FATTY ACIDS; DIETARY THERAPY;
D O I
10.1007/BF00968719
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
Brain, liver, and adipose lipids were studied in the postmortem tissues of four adrenoleukodystrophy patients who had been treated with a mixture of glyceryl trioleate and trierucate oils (''Lorenzo's Oil'') and compared to 7 untreated ALD patients and 3 controls. The dietary therapy appeared to reduce the levels of saturated very long chain fatty acids in the plasma, adipose tissue and liver; in the brain they were reduced in only one of the four patients. While substantial amounts of erucic acid were present in some of the tissues even 12 months after therapy had been discontinued, the levels in brain did not exceed those in controls al any time. The failure of erucic acid to enter the brain in significant quantity may be a factor in the disappointing results of dietary therapy for adrenoleukodystrophy.
引用
收藏
页码:1073 / 1082
页数:10
相关论文
共 40 条
[31]  
SCOTT BL, 1989, P NATL ACAD SCI USA, V86, P2093
[32]  
SIMOPOULOS AP, 1989, DIETARY OMEGA 3 OMEG, P391
[33]   TRENDS IN INDIVIDUAL CONSUMPTION OF DIETARY-FAT IN THE UNITED-STATES, 1920-1984 [J].
STEPHEN, AM ;
WALD, NJ .
AMERICAN JOURNAL OF CLINICAL NUTRITION, 1990, 52 (03) :457-469
[34]  
TAKETOMI T, 1987, JPN J EXP MED, V57, P59
[35]   PHOSPHOLIPIDS IN X-LINKED ADRENOLEUKODYSTROPHY WHITE MATTER - FATTY-ACID ABNORMALITIES BEFORE THE ONSET OF DEMYELINATION [J].
THEDA, C ;
MOSER, AB ;
POWERS, JM ;
MOSER, HW .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 1992, 110 (1-2) :195-204
[36]   METABOLISM OF [17,18-H-3(2)]HEXACOSANOIC ACID AND [15,16-H-3(2)]LIGNOCERIC ACID IN CULTURED SKIN FIBROBLASTS FROM PATIENTS WITH ADRENOLEUKODYSTROPHY (ALD) AND ADRENOMYELONEUROPATHY (AMN) [J].
TSUJI, S ;
SANOKAWAMURA, T ;
ARIGA, T ;
MIYATAKE, T .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 1985, 71 (2-3) :359-367
[37]  
UZIEL G, 1990, INT CONGR SER, V898, P163
[38]  
VANCE DE, 1967, J LIPID RES, V8, P621
[39]   THE DESIGN OF A DIET RESTRICTED IN SATURATED VERY LONG-CHAIN FATTY-ACIDS - THERAPEUTIC APPLICATION IN ADRENOLEUKODYSTROPHY [J].
VANDUYN, MA ;
MOSER, AE ;
BROWN, FR ;
SACKTOR, N ;
LIU, A ;
MOSER, HW .
AMERICAN JOURNAL OF CLINICAL NUTRITION, 1984, 40 (02) :277-284
[40]   LORENZO OIL AND THROMBOCYTOPENIA IN PATIENTS WITH ADRENOLEUKODYSTROPHY [J].
ZINKHAM, WH ;
KICKLER, T ;
BOREL, J ;
MOSER, HW .
NEW ENGLAND JOURNAL OF MEDICINE, 1993, 328 (15) :1126-1127