BIOCHEMICAL NATURE OF PYRUVATE-DEHYDROGENASE COMPLEX IN THE PATIENT WITH PRIMARY LACTIC ACIDEMIA

被引：4

作者：

KITANO, A

ENDO, F

KURODA, Y

ASO, S

KAWASAKI, T

MATSUDA, I

机构：

[1] UNIV TOKUSHIMA,SCH MED,DEPT PEDIAT,TOKUSHIMA 770,JAPAN

[2] JAPAN RED CROSS,MED CTR,DEPT PEDIAT,TOKYO 150,JAPAN

来源：

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

关键词：

D O I：

10.1007/BF01802031

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

引用

页码：379 / 385

页数：7

共 14 条

[1]

BLASS JP, 1983, METABOLIC BASIS INHE, P193

[2] HYPERAMMONEMIA AND LACTIC-ACIDOSIS IN A PATIENT WITH PYRUVATE-DEHYDROGENASE DEFICIENCY [J].

BROWN, GK ;

SCHOLEM, RD ;

HUNT, SM ;

HARRISON, JR ;

POLLARD, AC .

JOURNAL OF INHERITED METABOLIC DISEASE, 1987, 10 (04) :359-366

[3] LOW IMMUNOGENICITY OF THE COMMON LIPOAMIDE DEHYDROGENASE SUBUNIT (E3) OF MAMMALIAN PYRUVATE-DEHYDROGENASE AND 2-OXOGLUTARATE DEHYDROGENASE MULTIENZYME COMPLEXES [J].

DEMARCUCCI, OL ;

HUNTER, A ;

LINDSAY, JG .

BIOCHEMICAL JOURNAL, 1985, 226 (02) :509-517

[4] DEFICIENCY OF THE PYRUVATE-DEHYDROGENASE COMPONENT IN PYRUVATE-DEHYDROGENASE COMPLEX DEFICIENT HUMAN-FIBROBLASTS - IMMUNOLOGICAL IDENTIFICATION [J].

HO, L ;

HU, CWC ;

PACKMAN, S ;

PATEL, MS .

JOURNAL OF CLINICAL INVESTIGATION, 1986, 78 (03) :844-847

[5]

JILKA JM, 1986, J BIOL CHEM, V261, P1858

[6] SYSTEMIC DEFICIENCY OF THE 1ST COMPONENT OF THE PYRUVATE-DEHYDROGENASE COMPLEX [J].

KERR, DS ;

HO, L ;

BERLIN, CM ;

LANOUE, KF ;

TOWFIGHI, J ;

HOPPEL, CL ;

LUSK, MM ;

GONDEK, CM ;

PATEL, MS .

PEDIATRIC RESEARCH, 1987, 22 (03) :312-318

[7] MUTATION OF THE E1-ALPHA SUBUNIT OF THE PYRUVATE-DEHYDROGENASE COMPLEX, IN RELATION TO HETEROGENEITY [J].

KITANO, A ;

ENDO, F ;

MATSUDA, I ;

MIYABAYASHI, S ;

DAHL, HHM .

JOURNAL OF INHERITED METABOLIC DISEASE, 1989, 12 (02) :97-107

[8] PURIFICATION AND IMMUNOCHEMICAL STUDIES OF PYRUVATE-DEHYDROGENASE COMPLEX FROM RAT-HEART, AND CELL-FREE SYNTHESIS OF LIPOAMIDE DEHYDROGENASE, A COMPONENT OF THE COMPLEX [J].

MATUDA, S ;

SHIRAHAMA, T ;

SAHEKI, T ;

MIURA, S ;

MORI, M .

BIOCHIMICA ET BIOPHYSICA ACTA, 1983, 741 (01) :86-93

[9]

OFARRELL PH, 1975, J BIOL CHEM, V250, P4007

[10]

REED LJ, 1980, ENZYME REGULATION ME, P47

← 1 2 →