LIVER-TRANSPLANTATION TO PROVIDE LOW-DENSITY-LIPOPROTEIN RECEPTORS AND LOWER PLASMA-CHOLESTEROL IN A CHILD WITH HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA

被引:368
作者
BILHEIMER, DW
GOLDSTEIN, JL
GRUNDY, SM
STARZL, TE
BROWN, MS
机构
[1] UNIV TEXAS,HLTH SCI CTR,SW MED SCH,DEPT MOLEC GENET,DALLAS,TX 75235
[2] UNIV PITTSBURGH,CTR HLTH,DEPT SURG,PITTSBURGH,PA 15260
关键词
D O I
10.1056/NEJM198412273112603
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
引用
收藏
页码:1658 / 1664
页数:7
相关论文
共 29 条
[1]  
BERMAN M, DHEW NIH78180 PUBL
[2]   REDUCTION IN CHOLESTEROL AND LOW-DENSITY LIPOPROTEIN SYNTHESIS AFTER PORTACAVAL-SHUNT SURGERY IN A PATIENT WITH HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA [J].
BILHEIMER, DW ;
GOLDSTEIN, JL ;
GRUNDY, SM ;
BROWN, MS .
JOURNAL OF CLINICAL INVESTIGATION, 1975, 56 (06) :1420-1430
[3]   MEVINOLIN AND COLESTIPOL STIMULATE RECEPTOR-MEDIATED CLEARANCE OF LOW-DENSITY LIPOPROTEIN FROM PLASMA IN FAMILIAL HYPERCHOLESTEROLEMIA HETEROZYGOTES [J].
BILHEIMER, DW ;
GRUNDY, SM ;
BROWN, MS ;
GOLDSTEIN, JL .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1983, 80 (13) :4124-4128
[4]   METABOLIC STUDIES IN FAMILIAL HYPERCHOLESTEROLEMIA - EVIDENCE FOR A GENE-DOSAGE EFFECT INVIVO [J].
BILHEIMER, DW ;
STONE, NJ ;
GRUNDY, SM .
JOURNAL OF CLINICAL INVESTIGATION, 1979, 64 (02) :524-533
[5]   LIPOPROTEIN RECEPTORS IN THE LIVER - CONTROL SIGNALS FOR PLASMA-CHOLESTEROL TRAFFIC [J].
BROWN, MS ;
GOLDSTEIN, JL .
JOURNAL OF CLINICAL INVESTIGATION, 1983, 72 (03) :743-747
[6]   LOWERING PLASMA-CHOLESTEROL BY RAISING LDL RECEPTORS [J].
BROWN, MS ;
GOLDSTEIN, JL .
NEW ENGLAND JOURNAL OF MEDICINE, 1981, 305 (09) :515-517
[7]  
CUMMINGS RD, 1983, J BIOL CHEM, V258
[8]   ASSIGNMENT OF THE HUMAN-GENE FOR THE LOW-DENSITY LIPOPROTEIN RECEPTOR TO CHROMOSOME-19 - SYNTENY OF A RECEPTOR, A LIGAND, AND A GENETIC-DISEASE [J].
FRANCKE, U ;
BROWN, MS ;
GOLDSTEIN, JL .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1984, 81 (09) :2826-2830
[9]   GENETIC-HETEROGENEITY IN FAMILIAL HYPERCHOLESTEROLEMIA - EVIDENCE FOR 2 DIFFERENT MUTATIONS AFFECTING FUNCTIONS OF LOW-DENSITY LIPOPROTEIN RECEPTOR [J].
GOLDSTEIN, JL ;
DANA, SE ;
BRUNSCHEDE, GY ;
BROWN, MS .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1975, 72 (03) :1092-1096
[10]  
GOLDSTEIN JL, 1983, METHOD ENZYMOL, V98, P241