COPPER DEFICIENCY IN THE MITOCHONDRIA OF CULTURED SKIN FIBROBLASTS FROM PATIENTS WITH MENKES SYNDROME

被引：32

作者：

KODAMA, H

OKABE, I

YANAGISAWA, M

KODAMA, Y

机构：

[1] JICHI MED SCH,DEPT PEDIAT,MINAMI KAWACHI,TOCHIGI 32904,JAPAN

[2] TOKYO UNIV,OCEAN RES INST,NAKANO KU,TOKYO 164,JAPAN

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1989年 / 12卷 / 04期

关键词：

D O I：

10.1007/BF01802032

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

引用

页码：386 / 389

页数：4

共 13 条

[1] ALTERED COPPER-METABOLISM IN CULTURED-CELLS FROM HUMAN MENKES SYNDROME AND MOTTLED MOUSE MUTANTS [J].

CAMAKARIS, J ;

DANKS, DM ;

ACKLAND, L ;

CARTWRIGHT, E ;

BORGER, P ;

COTTON, RGH .

BIOCHEMICAL GENETICS, 1980, 18 (1-2) :117-131

[2] CELL-CULTURE STUDIES OF MENKES KINKY HAIR DISEASE [J].

CHAN, WY ;

GARNICA, AD ;

RENNERT, OM .

CLINICA CHIMICA ACTA, 1978, 88 (03) :495-507

[3] MENKES KINKY HAIR DISEASE - FURTHER DEFINITION OF DEFECT IN COPPER TRANSPORT [J].

DANKS, DM ;

CARTWRIGHT, E ;

STEVENS, BJ ;

TOWNLEY, RRW .

SCIENCE, 1973, 179 (4078) :1140-1142

[4]

GLASER JH, 1973, J LAB CLIN MED, V82, P969

[5] MENKES DISEASE - ABNORMAL METALLOTHIONEIN GENE-REGULATION IN RESPONSE TO COPPER [J].

LEONE, A ;

PAVLAKIS, GN ;

HAMER, DH .

CELL, 1985, 40 (02) :301-309

[6]

LOWRY OH, 1951, J BIOL CHEM, V193, P265

[7] BIOCHEMICAL-STUDY IN 28 CHILDREN WITH LACTIC-ACIDOSIS, IN RELATION TO LEIGHS ENCEPHALOMYELOPATHY [J].

MIYABAYASHI, S ;

ITO, T ;

NARISAWA, K ;

IINUMA, K ;

TADA, K .

EUROPEAN JOURNAL OF PEDIATRICS, 1985, 143 (04) :278-283

[8] COPPER UTILIZATION IN CULTURED SKIN FIBROBLASTS OF THE MOTTLED MOUSE, AN ANIMAL-MODEL FOR MENKES KINKY HAIR SYNDROME [J].

PACKMAN, S ;

CHIN, P ;

OTOOLE, C .

JOURNAL OF INHERITED METABOLIC DISEASE, 1984, 7 (04) :168-170

[9] METALLOTHIONEIN MESSENGER-RNA REGULATION IN THE MOTTLED MOUSE AND MENKES KINKY HAIR SYNDROME [J].

PACKMAN, S ;

PALMITER, RD ;

KARIN, M ;

OTOOLE, C .

JOURNAL OF CLINICAL INVESTIGATION, 1987, 79 (05) :1338-1342

[10] DEMONSTRATION OF A SPECIFIC MITOCHONDRIAL ISOVALERYL-COA DEHYDROGENASE-DEFICIENCY IN FIBROBLASTS FROM PATIENTS WITH ISOVALERIC ACIDEMIA [J].

RHEAD, WJ ;

TANAKA, K .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1980, 77 (01) :580-583

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