139 related articles for article (PubMed ID: 6414741)
1. Revised assays for the investigation of congenital lactic acidosis using 14C keto acids, eliminating problems associated with spontaneous decarboxylation.
Hyland K; Leonard JV
Clin Chim Acta; 1983 Sep; 133(2):177-87. PubMed ID: 6414741
[TBL] [Abstract][Full Text] [Related]
2. Absence of pyruvate decarboxylase activity in man: a cause of congenital lactic acidosis.
Farrell DF; Clark AF; Scott CR; Wennberg RP
Science; 1975 Mar; 187(4181):1082-4. PubMed ID: 803713
[TBL] [Abstract][Full Text] [Related]
3. Abnormal pyruvate and alpha-ketoglutarate dehydrogenase complexes in a patient with lactic acidemia.
Kuroda Y; Kline JJ; Sweetman L; Nyhan WL; Groshong TD
Pediatr Res; 1979 Aug; 13(8):928-31. PubMed ID: 481967
[TBL] [Abstract][Full Text] [Related]
4. Pyruvate-dehydrogenase complex in ataxic patients: enzyme deficiency in ataxic encephalopathy plus lactic acidosis and normal activity in Friedreich ataxia.
Uziel G; Bottacchi E; Moschen G; Giovanardi-Rossi P; Cardace G; Di Donato S
Ital J Neurol Sci; 1982 Dec; 3(4):317-21. PubMed ID: 6820014
[TBL] [Abstract][Full Text] [Related]
5. Deficiency of dihydrolipoyl dehydrogenase (a component of the pyruvate and alpha-ketoglutarate dehydrogenase complexes): a cause of congenital chronic lactic acidosis in infancy.
Robinson BH; Taylor J; Sherwood WG
Pediatr Res; 1977 Dec; 11(12):1198-202. PubMed ID: 413089
[TBL] [Abstract][Full Text] [Related]
6. Inhibition of glycine oxidation by pyruvate, alpha-ketoglutarate, and branched-chain alpha-keto acids in rat liver mitochondria: presence of interaction between the glycine cleavage system and alpha-keto acid dehydrogenase complexes.
Kochi H; Seino H; Ono K
Arch Biochem Biophys; 1986 Sep; 249(2):263-72. PubMed ID: 3753002
[TBL] [Abstract][Full Text] [Related]
7. Congenital lactic acidosis, alpha-ketoglutaric aciduria and variant form of maple syrup urine disease due to a single enzyme defect: dihydrolipoyl dehydrogenase deficiency.
Munnich A; Saudubray JM; Taylor J; Charpentier C; Marsac C; Rocchiccioli F; Amedee-Manesme O; Coude FX; Frezal J; Robinson BH
Acta Paediatr Scand; 1982 Jan; 71(1):167-71. PubMed ID: 6897145
[TBL] [Abstract][Full Text] [Related]
8. Effect of lipoic acid in a patient with defective activity of pyruvate dehydrogenase, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase.
Yoshida I; Sweetman L; Kulovich S; Nyhan WL; Robinson BH
Pediatr Res; 1990 Jan; 27(1):75-9. PubMed ID: 2104971
[TBL] [Abstract][Full Text] [Related]
9. Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria.
Matuda S; Kitano A; Sakaguchi Y; Yoshino M; Saheki T
Clin Chim Acta; 1984 Jun; 140(1):59-64. PubMed ID: 6430599
[TBL] [Abstract][Full Text] [Related]
10. A defect in branched-chain amino acid metabolism in a patient with congenital lactic acidosis due to dihydrolipoyl dehydrogenase deficiency.
Taylor J; Robinson BH; Sherwood WG
Pediatr Res; 1978 Jan; 12(1):60-2. PubMed ID: 643378
[TBL] [Abstract][Full Text] [Related]
11. [Chronic infantile lactate acidosis].
Papanastasiou D; Lehnert W; Schuchmann L; Hommes FA
Helv Paediatr Acta; 1980 Jul; 35(3):253-60. PubMed ID: 7410111
[TBL] [Abstract][Full Text] [Related]
12. The genetic heterogeneity of lactic acidosis: occurrence of recognizable inborn errors of metabolism in pediatric population with lactic acidosis.
Robinson BH; Taylor J; Sherwood WG
Pediatr Res; 1980 Aug; 14(8):956-62. PubMed ID: 6775276
[TBL] [Abstract][Full Text] [Related]
13. Tyrphostin 47 nonenzymatically decarboxylates [1-14C]-pyruvate.
Kiechle FL; Staudacher DM; Ofenstein JP
Ann Clin Lab Sci; 1994; 24(5):422-30. PubMed ID: 7818237
[TBL] [Abstract][Full Text] [Related]
14. Problems in the congenital lactic acidoses.
Leonard JV
Ciba Found Symp; 1982; 87():340-56. PubMed ID: 6280937
[TBL] [Abstract][Full Text] [Related]
15. Molecular analysis of abnormal pyruvate dehydrogenase in a patient with thiamine-responsive congenital lactic acidemia.
Naito E; Ito M; Takeda E; Yokota I; Yoshijima S; Kuroda Y
Pediatr Res; 1994 Sep; 36(3):340-6. PubMed ID: 7808831
[TBL] [Abstract][Full Text] [Related]
16. Determination of pyruvate dehydrogenase in cultured human fibroblasts and amniotic fluid cells.
Hansen TL
Clin Chim Acta; 1982 Aug; 123(1-2):45-50. PubMed ID: 6811160
[TBL] [Abstract][Full Text] [Related]
17. Demonstration of an unstable variant of pyruvate dehydrogenase protein (E1) in cultured fibroblasts from a patient with congenital lactic acidemia.
Huq AH; Ito M; Naito E; Saijo T; Takeda E; Kuroda Y
Pediatr Res; 1991 Jul; 30(1):11-4. PubMed ID: 1909778
[TBL] [Abstract][Full Text] [Related]
18. Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase.
Strömme JH; Borud O; Moe PJ
Pediatr Res; 1976 Jan; 10(1):62-6. PubMed ID: 813176
[TBL] [Abstract][Full Text] [Related]
19. Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts.
Sheu KF; Hu CW; Utter MF
J Clin Invest; 1981 May; 67(5):1463-71. PubMed ID: 6262377
[TBL] [Abstract][Full Text] [Related]
20. Organic acids in urine of patients with congenital lactic acidoses: an aid to differential diagnosis.
Chalmers RA
J Inherit Metab Dis; 1984; 7 Suppl 1():79-89. PubMed ID: 6434850
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]