CHRONIC ACIDEMIA DUE TO A PYRUVATE-DEHYDROGENASE DEFICIENCY IN THE PYRUVATE-DEHYDROGENASE COMPLEX, WITH EVIDENCE OF ABNORMALITIES OF THE ALPHA-SUBUNIT AND BETA-SUBUNIT OF THE ENZYME

被引：0
|
作者：
KOIKE, K
URATA, Y
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：450 / 452
页数：3
相关论文
共 50 条
  • [31] PYRUVATE-DEHYDROGENASE COMPLEX DEFICIENCY EXPRESSED IN LYMPHOCYTES
    KERR, DS
    LUSK, MM
    PEDIATRIC RESEARCH, 1985, 19 (04) : A314 - A314
  • [32] PROPERTIES AND SUBUNIT STRUCTURE OF PIG HEART PYRUVATE-DEHYDROGENASE
    HAMADA, M
    HIRAOKA, T
    KOIKE, K
    OGASAHARA, K
    KANZAKI, T
    KOIKE, M
    JOURNAL OF BIOCHEMISTRY, 1976, 79 (06): : 1273 - 1285
  • [33] DEFICIENCY OF THE ALPHA-SUBUNIT AND BETA-SUBUNITS OF PYRUVATE-DEHYDROGENASE IN A PATIENT WITH LACTIC-ACIDOSIS AND UNEXPECTED SUDDEN-DEATH
    SPERL, W
    RUITENBEEK, W
    KERKHOF, CMC
    SENGERS, RCA
    TRIJBELS, JMF
    GUGGENBICHLER, JP
    JANSSEN, AJM
    BAKKEREN, JAJM
    EUROPEAN JOURNAL OF PEDIATRICS, 1990, 149 (07) : 487 - 492
  • [34] MUTATION OF THE E1-ALPHA SUBUNIT OF THE PYRUVATE-DEHYDROGENASE COMPLEX, IN RELATION TO HETEROGENEITY
    KITANO, A
    ENDO, F
    MATSUDA, I
    MIYABAYASHI, S
    DAHL, HHM
    JOURNAL OF INHERITED METABOLIC DISEASE, 1989, 12 (02) : 97 - 107
  • [35] MITOCHONDRIAL CYTOPATHY - PYRUVATE-DEHYDROGENASE COMPLEX DEFICIENCY
    MIYABAYASHI, S
    BRAIN & DEVELOPMENT, 1987, 9 (02): : 85 - 86
  • [36] THE A-SUUM PYRUVATE-DEHYDROGENASE COMPLEX HAS A NOVEL SUBUNIT COMPOSITION
    KOMUNIECKI, R
    RHEE, B
    BHAT, D
    SONG, H
    DURAN, E
    FASEB JOURNAL, 1991, 5 (05): : A1202 - A1202
  • [37] SUBUNIT BINDING IN THE PYRUVATE-DEHYDROGENASE COMPLEX FROM BOVINE KIDNEY AND HEART
    WU, TL
    REED, LJ
    BIOCHEMISTRY, 1984, 23 (02) : 221 - 226
  • [38] ARGININE-239 IN THE BETA-SUBUNIT IS AT OR NEAR THE ACTIVE-SITE OF BOVINE PYRUVATE-DEHYDROGENASE
    ESWARAN, D
    ALI, MS
    SHENOY, BC
    KOROTCHKINA, LG
    ROCHE, TE
    PATEL, MS
    BIOCHIMICA ET BIOPHYSICA ACTA-PROTEIN STRUCTURE AND MOLECULAR ENZYMOLOGY, 1995, 1252 (02): : 203 - 208
  • [39] INFLUENCE OF SUBUNIT CROSS-LINKING ON COOPERATIVITY OF PYRUVATE-DEHYDROGENASE COMPLEX
    BISSWANGER, H
    HOPPE-SEYLERS ZEITSCHRIFT FUR PHYSIOLOGISCHE CHEMIE, 1977, 358 (03): : 215 - 215
  • [40] CHARACTERIZATION OF THE PROMOTER REGION OF THE BETA-SUBUNIT OF THE HUMAN PYRUVATE-DEHYDROGENASE (E1) GENE
    NAIK, SS
    MADHUSUDHAN, KT
    PATEL, MS
    FASEB JOURNAL, 1993, 7 (07): : A1220 - A1220
12345