DEFICIENT ACTIVITIES AND PROTEINS OF PEROXISOMAL BETA-OXIDATION ENZYMES IN INFANTS WITH ZELLWEGER SYNDROME

被引:38
作者
SUZUKI, Y
ORII, T
MORI, M
TATIBANA, M
HASHIMOTO, T
机构
[1] KUMAMOTO UNIV,SCH MED,INST MED GENET,KUMAMOTO 862,JAPAN
[2] CHIBA UNIV,SCH MED,DEPT BIOCHEM,CHIBA 280,JAPAN
[3] SHINSHU UNIV,FAC MED,DEPT BIOCHEM,MATSUMOTO,NAGANO 390,JAPAN
关键词
D O I
10.1016/0009-8981(86)90152-X
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
引用
收藏
页码:191 / 196
页数:6
相关论文
共 23 条
  • [1] THE CEREBRO-HEPATO-RENAL (ZELLWEGER) SYNDROME - LAMELLAR LIPID PROFILES IN ADRENOCORTICAL, HEPATIC MESENCHYMAL, ASTROCYTE CELLS AND INCREASED LEVELS OF VERY LONG-CHAIN FATTY-ACIDS AND PHYTANIC ACID IN THE PLASMA
    AUBOURG, P
    ROBAIN, O
    ROCCHICCIOLI, F
    DANCEA, S
    SCOTTO, J
    [J]. JOURNAL OF THE NEUROLOGICAL SCIENCES, 1985, 69 (1-2) : 9 - 25
  • [2] BOWEN P, 1964, B JOHNS HOPKINS HOSP, V114, P402
  • [3] BROWN FR, 1982, JOHNS HOPKINS MED J, V151, P344
  • [4] CEREBRO-HEPATO-RENAL SYNDROME OF ZELLWEGER - REPORT OF 8 CASES WITH COMMENTS UPON INCIDENCE, LIVER LESION, AND A FAULT IN PIPECOLIC ACID METABOLISM
    DANKS, DM
    TIPPETT, P
    ADAMS, C
    CAMPBELL, P
    [J]. JOURNAL OF PEDIATRICS, 1975, 86 (03) : 382 - 387
  • [5] PROPERTIES OF MITOCHONDRIAL AND PEROXISOMAL ENOYL-COA HYDRATASES FROM RAT-LIVER
    FURUTA, S
    MIYAZAWA, S
    OSUMI, T
    HASHIMOTO, T
    UI, N
    [J]. JOURNAL OF BIOCHEMISTRY, 1980, 88 (04) : 1059 - 1070
  • [6] PEROXISOMAL AND MITOCHONDRIAL DEFECTS IN CEREBRO-HEPATO-RENAL SYNDROME
    GOLDFISCHER, S
    MOORE, CL
    JOHNSON, AB
    SPIRO, AJ
    VALSAMIS, MP
    WISNIEWSKI, HK
    RITCH, RH
    NORTON, WT
    RAPIN, I
    GARTNER, LM
    [J]. SCIENCE, 1973, 182 (4107) : 62 - 64
  • [7] PEROXISOMAL DEFECTS IN NEONATAL-ONSET AND X-LINKED ADRENOLEUKODYSTROPHIES
    GOLDFISCHER, S
    COLLINS, J
    RAPIN, I
    COLTOFFSCHILLER, B
    CHANG, CH
    NIGRO, M
    BLACK, VH
    JAVITT, NB
    MOSER, HW
    LAZAROW, PB
    [J]. SCIENCE, 1985, 227 (4682) : 67 - 70
  • [8] DEFECTS OF BILE-ACID SYNTHESIS IN ZELLWEGER SYNDROME
    HANSON, RF
    SZCZEPANIKVANLEEUWEN, P
    WILLIAMS, GC
    GRABOWSKI, G
    SHARP, HL
    [J]. SCIENCE, 1979, 203 (4385) : 1107 - 1108
  • [9] ALPHA-AMANITIN INHIBITS THE OXIDATION OF LONG-CHAIN FATTY-ACIDS IN MOUSE-LIVER
    HASHIMOTO, T
    MIYAZAWA, S
    GUNARSO, D
    FURUTA, S
    [J]. JOURNAL OF BIOCHEMISTRY, 1981, 90 (02) : 415 - 421
  • [10] INDIVIDUAL PEROXISOMAL BETA-OXIDATION ENZYMES
    HASHIMOTO, T
    [J]. ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, 1982, 386 (MAY) : 5 - 12