IMMUNOCHEMICAL STUDIES OF FIBROBLASTS FROM PATIENTS WITH METHYLMALONYL-COA MUTASE APOENZYME DEFICIENCY - DETECTION OF A MUTATION INTERFERING WITH MITOCHONDRIAL IMPORT

被引:32
作者
FENTON, WA
HACK, AM
KRAUS, JP
ROSENBERG, LE
机构
关键词
D O I
10.1073/pnas.84.5.1421
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
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页码:1421 / 1424
页数:4
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共 15 条
[1]   ISOLATION OF BIOLOGICALLY-ACTIVE RIBONUCLEIC-ACID FROM SOURCES ENRICHED IN RIBONUCLEASE [J].
CHIRGWIN, JM ;
PRZYBYLA, AE ;
MACDONALD, RJ ;
RUTTER, WJ .
BIOCHEMISTRY, 1979, 18 (24) :5294-5299
[2]   PROTEIN IMPORT INTO ORGANELLES - HIERARCHICAL TARGETING SIGNALS [J].
COLMAN, A ;
ROBINSON, C .
CELL, 1986, 46 (03) :321-322
[3]   TARGETING PROTEINS INTO MITOCHONDRIA [J].
DOUGLAS, MG ;
MCCAMMON, MT ;
VASSAROTTI, A .
MICROBIOLOGICAL REVIEWS, 1986, 50 (02) :166-178
[4]  
FENTON WA, 1984, J BIOL CHEM, V259, P6616
[5]  
GEAR ARL, 1974, J BIOL CHEM, V249, P3628
[6]   TARGETING OF PRE-ORNITHINE TRANSCARBAMYLASE TO MITOCHONDRIA - DEFINITION OF CRITICAL REGIONS AND RESIDUES IN THE LEADER PEPTIDE [J].
HORWICH, AL ;
KALOUSEK, F ;
FENTON, WA ;
POLLOCK, RA ;
ROSENBERG, LE .
CELL, 1986, 44 (03) :451-459
[7]   EXPRESSION OF AMPLIFIED DNA-SEQUENCES FOR ORNITHINE TRANSCARBAMYLASE IN HELA-CELLS - ARGININE RESIDUES MAY BE REQUIRED FOR MITOCHONDRIAL IMPORT OF ENZYME PRECURSORS [J].
HORWICH, AL ;
FENTON, WA ;
FIRGAIRA, FA ;
FOX, JE ;
KOLANSKY, D ;
MELLMAN, IS ;
ROSENBERG, LE .
JOURNAL OF CELL BIOLOGY, 1985, 100 (05) :1515-1521
[8]   A LEADER PEPTIDE IS SUFFICIENT TO DIRECT MITOCHONDRIAL IMPORT OF A CHIMERIC PROTEIN [J].
HORWICH, AL ;
KALOUSEK, F ;
MELLMAN, I ;
ROSENBERG, LE .
EMBO JOURNAL, 1985, 4 (05) :1129-1135
[9]   BIOSYNTHESIS OF VARIANT MEDIUM CHAIN ACYL-COA DEHYDROGENASE IN CULTURED FIBROBLASTS FROM PATIENTS WITH MEDIUM CHAIN ACYL-COA DEHYDROGENASE-DEFICIENCY [J].
IKEDA, Y ;
HALE, DE ;
KEESE, SM ;
COATES, PM ;
TANAKA, K .
PEDIATRIC RESEARCH, 1986, 20 (09) :843-847
[10]   MOLECULAR HETEROGENEITY OF VARIANT ISOVALERYL-COA DEHYDROGENASE FROM CULTURED ISOVALERIC ACIDEMIA FIBROBLASTS [J].
IKEDA, Y ;
KEESE, SM ;
TANAKA, K .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1985, 82 (20) :7081-7085