CDNA CLONING OF THE E1-ALPHA SUBUNIT OF THE BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE AND ELUCIDATION OF A MOLECULAR-BASIS FOR MAPLE SYRUP URINE DISEASE

被引：0
作者：
ZHANG, B
KUNTZ, MJ
GOODWIN, GW
EDENBERG, HJ
CRABB, DW
HARRIS, RA
机构：
来源：
ALPHA-KETO ACID DEHYDROGENASE COMPLEXES : ORGANIZATION, REGULATION, AND BIOMEDICAL RAMIFICATIONS: A TRIBUTE TO LESTER J REED | 1989年 / 573卷
关键词：
D O I：
暂无
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
收藏
页码：130 / 136
页数：7
相关论文
共 50 条
[41]   MOLECULAR-CLONING OF A CDNA FOR THE E1-ALPHA-SUBUNIT OF RAT-LIVER BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE [J].
ZHANG, B ;
KUNTZ, MJ ;
GOODWIN, GW ;
HARRIS, RA ;
CRABB, DW .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1987, 262 (31) :15220-15224
[42]   ISOLATION OF A BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE FROM RABBIT LIVER [J].
MORRISON, EYS ;
MULLINGS, K .
BIOCHIMICA ET BIOPHYSICA ACTA, 1983, 755 (02) :225-228
[43]   BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE COMPLEX FROM BOVINE KIDNEY [J].
PETTIT, FH ;
REED, LJ .
METHODS IN ENZYMOLOGY, 1988, 166 :309-312
[44]   HIGH BRANCHED-CHAIN ALPHA-KETO ACID INTAKE, BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE-ACTIVITY, AND PLASMA AND BRAIN AMINO-ACID AND PLASMA KETO ACID CONCENTRATIONS IN RATS [J].
CROWELL, PL ;
BLOCK, KP ;
REPA, JJ ;
TORRES, N ;
NAWABI, MD ;
BUSE, MG ;
HARPER, AE .
AMERICAN JOURNAL OF CLINICAL NUTRITION, 1990, 52 (02) :313-319
[45]   PREMATURE TRANSLATION TERMINATION OF THE PRE-E1-ALPHA-SUBUNIT OF THE BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE AS A CAUSE OF MAPLE SYRUP URINE DISEASE IN POLLED HEREFORD CALVES [J].
ZHANG, B ;
HEALY, PJ ;
ZHAO, Y ;
CRABB, DW ;
HARRIS, RA .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1990, 265 (05) :2425-2427
[46]   DEVELOPMENT OF AN ANIMAL-MODEL FOR MAPLE SYRUP URINE DISEASE BY DIETARY BRANCHED-CHAIN AMINO-ACIDS AND TREATMENT WITH BETA-CHLOROALANINE, AN ALPHA-KETO ACID DEHYDROGENASE INHIBITOR [J].
DWIVEDI, C ;
KRIEGER, JA ;
MUNGER, KD .
BIOCHEMICAL ARCHIVES, 1992, 8 (02) :81-85
[47]   STRUCTURAL ORGANIZATION AND CHROMOSOMAL LOCALIZATION OF THE GENE FOR THE E1-BETA SUBUNIT OF HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE [J].
MITSUBUCHI, H ;
NOBUKUNI, Y ;
ENDO, F ;
MATSUDA, I .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1991, 266 (22) :14686-14691
[48]   SEQUENCE CONSERVATION IN THE ALPHA-SUBUNIT AND BETA-SUBUNIT OF PYRUVATE-DEHYDROGENASE AND ITS SIMILARITY TO BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE [J].
WEXLER, ID ;
HEMALATHA, SG ;
PATEL, MS .
FEBS LETTERS, 1991, 282 (01) :209-213
[49]   HETEROGENEITY OF MUTATIONS IN MAPLE-SYRUP-URINE-DISEASE (MSUD) - SCREENING AND IDENTIFICATION OF AFFECTED E1-ALPHA AND E1-BETA SUBUNITS OF THE BRANCHED-CHAIN ALPHA-KETO-ACID DEHYDROGENASE MULTIENZYME COMPLEX (VOL 1225, PG 64, 1993) [J].
NOBUKUNI, Y ;
MITSUBUCHI, H ;
HAGASHIDA, Y ;
OHTA, K ;
INDO, Y ;
ICHIBA, Y ;
ENDO, F ;
MATSUDA, I .
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, 1994, 1226 (01) :115-115
[50]   THE COMPLETE CDNA SEQUENCE FOR DIHYDROLIPOYL TRANSACYLASE (E2) OF HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE COMPLEX [J].
LAU, KS ;
CHUANG, JL ;
HERRING, WJ ;
DANNER, DJ ;
COX, RP ;
CHUANG, DT .
BIOCHIMICA ET BIOPHYSICA ACTA, 1992, 1132 (03) :319-321
12345