MOLECULAR ANALYSIS OF PEROXISOMAL BETA-OXIDATION ENZYMES IN INFANTS WITH PEROXISOMAL DISORDERS INDICATES HETEROGENEITY OF THE PRIMARY DEFECT

被引：12

作者：

GUERROUI, S

AUBOURG, P

CHEN, WW

HASHIMOTO, T

SCOTTO, J

机构：

[1] HOP ST VINCENT PAUL,INSERM,U188,F-75674 PARIS 14,FRANCE

[2] JOHNS HOPKINS UNIV,SCH MED,KENNEDY INST,BALTIMORE,MD 21205

[3] JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROL,BALTIMORE,MD 21205

[4] SHINSHU UNIV,SCH MED,DEPT BIOCHEM,MATSUMOTO,NAGANO 390,JAPAN

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1989年 / 161卷 / 01期

关键词：

D O I：

10.1016/0006-291X(89)91587-8

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

页码：242 / 251

页数：10

共 36 条

[11] NEONATAL ADRENOLEUKODYSTROPHY - NEW CASES, BIOCHEMICAL-STUDIES, AND DIFFERENTIATION FROM ZELLWEGER AND RELATED PEROXISOMAL POLYDYSTROPHY SYNDROMES [J].

KELLEY, RI ;

DATTA, NS ;

DOBYNS, WB ;

HAJRA, AK ;

MOSER, AB ;

NOETZEL, MJ ;

ZACKAI, EH ;

MOSER, HW .

AMERICAN JOURNAL OF MEDICAL GENETICS, 1986, 23 (04) :869-901

[12] PROPERTIES OF PEROXISOMAL 3-KETOACYL-COA THIOLASE FROM RAT-LIVER [J].

MIYAZAWA, S ;

FURUTA, S ;

OSUMI, T ;

HASHIMOTO, T ;

UI, N .

JOURNAL OF BIOCHEMISTRY, 1981, 90 (02) :511-519

[13] PEROXISOMAL DISORDERS - CLINICAL CHARACTERIZATION [J].

MONNENS, L ;

HEYMANS, H .

JOURNAL OF INHERITED METABOLIC DISEASE, 1987, 10 :23-32

[14] THE CEREBROHEPATORENAL (ZELLWEGER) SYNDROME - INCREASED LEVELS AND IMPAIRED DEGRADATION OF VERY-LONG-CHAIN FATTY-ACIDS AND THEIR USE IN PRENATAL-DIAGNOSIS [J].

MOSER, AE ;

SINGH, I ;

BROWN, FR ;

SOLISH, GI ;

KELLEY, RI ;

BENKE, PJ ;

MOSER, HW .

NEW ENGLAND JOURNAL OF MEDICINE, 1984, 310 (18) :1141-1146

[15]

MOSER HW, 1985, HOSP PRACT, V20, P61

[16] NEONATAL SEIZURES AND RETARDATION IN A GIRL WITH BIOCHEMICAL FEATURES OF X-LINKED ADRENOLEUKODYSTROPHY - A POSSIBLE NEW PEROXISOMAL DISEASE ENTITY [J].

NAIDU, S ;

HOEFLER, G ;

WATKINS, PA ;

CHEN, WW ;

MOSER, AB ;

HOEFLER, S ;

RANCE, NE ;

POWERS, JM ;

BEARD, M ;

GREEN, WR ;

HASHIMOTO, T ;

MOSER, HW .

NEUROLOGY, 1988, 38 (07) :1100-1107

[17] PURIFICATION AND PROPERTIES OF ACYL-COA OXIDASE FROM RAT-LIVER [J].

OSUMI, T ;

HASHIMOTO, T ;

UI, N .

JOURNAL OF BIOCHEMISTRY, 1980, 87 (06) :1735-1746

[18] PEROXISOMAL BETA-OXIDATION SYSTEM OF RAT-LIVER - COPURIFICATION OF ENOYL-COA HYDRATASE AND 3-HYDROXYACYL-COA DEHYDROGENASE [J].

OSUMI, T ;

HASHIMOTO, T .

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1979, 89 (02) :580-584

[19]

Osumi T., 1987, PEROXISOMES BIOL MED, P105

[20]

POLLTHE BT, 1988, AM J HUM GENET, V42, P422

← 1 2 3 4 →