HYPERAMMONEMIA AND LACTIC-ACIDOSIS IN A PATIENT WITH PYRUVATE-DEHYDROGENASE DEFICIENCY

被引：40

作者：

BROWN, GK

SCHOLEM, RD

HUNT, SM

HARRISON, JR

POLLARD, AC

机构：

[1] ADELAIDE CHILDRENS HOSP,DEPT CHEM PATHOL,N ADELAIDE,SA 5006,AUSTRALIA

[2] ROYAL CHILDRENS HOSP,MURDOCH INST RES BIRTH DEFECTS,MELBOURNE,AUSTRALIA

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1987年 / 10卷 / 04期

关键词：

D O I：

10.1007/BF01799978

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

引用

页码：359 / 366

页数：8

共 22 条

[1]

ADACHI TORU, 1963, J VITAMINOL, V9, P217

[2] PYRUVATE-CARBOXYLASE AND PHOSPHOENOLPYRUVATE CARBOXYKINASE ACTIVITY IN LEUKOCYTES AND FIBROBLASTS FROM A PATIENT WITH PYRUVATE-CARBOXYLASE DEFICIENCY [J].

ATKIN, BM ;

UTTER, MF ;

WEINBERG, MB .

PEDIATRIC RESEARCH, 1979, 13 (01) :38-43

[3] AMINO-ACID PROFILE IN PYRUVATE-CARBOXYLASE DEFICIENCY - COMPARISON WITH SOME OTHER METABOLIC DISORDERS [J].

CHARPENTIER, C ;

TETAU, JM ;

OGIER, H ;

SAUDUBRAY, JM ;

COUDE, FX ;

LEMONNIER, A .

JOURNAL OF INHERITED METABOLIC DISEASE, 1982, 5 :11-12

[4] INSITU DETECTION OF MYCOPLASMA CONTAMINATION IN CELL-CULTURES BY FLUORESCENT HOECHST-33258 STAIN [J].

CHEN, TR .

EXPERIMENTAL CELL RESEARCH, 1977, 104 (02) :255-262

[5] INHIBITION BY PROPIONYL-COENZYME-A OF N-ACETYLGLUTAMATE SYNTHETASE IN RAT-LIVER MITOCHONDRIA - POSSIBLE EXPLANATION FOR HYPER-AMMONEMIA IN PROPIONIC AND METHYLMALONIC ACIDEMIA [J].

COUDE, FX ;

SWEETMAN, L ;

NYHAN, WL .

JOURNAL OF CLINICAL INVESTIGATION, 1979, 64 (06) :1544-1551

[6]

COUDE FX, 1981, PEDIATRICS, V68, P914

[7]

DAHL HHM, 1987, IN PRESS J BIOL CHEM

[8] 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

[9] 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

[10]

LANDES RD, 1972, PEDIATR RES, V6, P394

← 1 2 3 →