103 related articles for article (PubMed ID: 630060)
1. Abnormal metabolites of isoleucine in a patient with propionyl-CoA carboxylase deficiency.
Sweetman L; Weyler W; Nyhan WL; de Céspedes C; Loria AR; Estrada Y
Biomed Mass Spectrom; 1978 Mar; 5(3):198-207. PubMed ID: 630060
[TBL] [Abstract][Full Text] [Related]
2. The identification of 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid in a patient with propionic acidemia.
Truscott RJ; Pullin CJ; Halpern B; Hammond J; Haan E; Danks DM
Biomed Mass Spectrom; 1979 Jul; 6(7):294-300. PubMed ID: 486715
[TBL] [Abstract][Full Text] [Related]
3. Propionyl-CoA carboxylase deficiency: case report, effect of low-protein diet and identification of 3-oxo-2-methylvaleric acid 3-hydroxy-2-methylvaleric acid, and maleic acid in urine.
Bergstrøm T; Greter J; Levin AH; Steen G; Tryding N; Wass U
Scand J Clin Lab Invest; 1981 Apr; 41(2):117-26. PubMed ID: 7313494
[TBL] [Abstract][Full Text] [Related]
4. Propionyl-CoA carboxylase deficiency in a patient with biotin-responsive 3-methylcrotonylglycinuria.
Sweetman L; Bates SP; Hull D; Nyhan WL
Pediatr Res; 1977 Nov; 11(11):1144-7. PubMed ID: 917614
[TBL] [Abstract][Full Text] [Related]
5. Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels.
Przyrembel H; Bremer HJ; Duran M; Bruinvis L; Ketting D; Wadman SK; Baumgartner R; Irle U; Bachmann C
Eur J Pediatr; 1979 Jan; 130(1):1-14. PubMed ID: 759179
[TBL] [Abstract][Full Text] [Related]
6. [Effects of propionic acid metabolic precursors in biotin-deprived rats].
Cherruau B; Mangeot M; Demelier JF; Charpentier C; Pelletier C; Lemonnier A
Nutr Metab; 1980; 24(6):367-82. PubMed ID: 7219898
[TBL] [Abstract][Full Text] [Related]
7. Dietary treatment and biochemical studies on a neonatal case of propionyl-CoA carboxylase deficiency.
DelValle JA; Merinero B; Jiménez A; García MJ; Ugarte M; Omeñaca F; Neustadt G; Quero J
J Inherit Metab Dis; 1982; 5(2):121-4. PubMed ID: 6820422
[TBL] [Abstract][Full Text] [Related]
8. The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with beta-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia.
Duran M; Bruinvis L; Ketting D; Kamerling JP; Wadman SK; Schutgens RB
Biomed Mass Spectrom; 1982 Jan; 9(1):1-5. PubMed ID: 7059658
[TBL] [Abstract][Full Text] [Related]
9. [2-Methyl-3-oxovaleric acid: a characteristic metabolite in propionic acidemia].
Lehnert W; Junker A
Clin Chim Acta; 1980 May; 104(1):47-51. PubMed ID: 7389125
[TBL] [Abstract][Full Text] [Related]
10. Urinary excretion of 2-methylacetoacetate, 2-methyl-3-hydroxybutyrate and tiglylglycine after isoleucine loading in the diagnosis of 2-methylacetoacetyl-CoA thiolase deficiency.
Aramaki S; Lehotay D; Sweetman L; Nyhan WL; Winter SC; Middleton B
J Inherit Metab Dis; 1991; 14(1):63-74. PubMed ID: 1861461
[TBL] [Abstract][Full Text] [Related]
11. The synthesis and characterisation of 2-methylacetoacetyl coenzyme A and its use in the identification of the site of the defect in 2-methylacetoacetic and 2-methyl-3-hydroxybutyric aciduria.
Middleton B; Bartlett K
Clin Chim Acta; 1983 Mar; 128(2-3):291-305. PubMed ID: 6133656
[TBL] [Abstract][Full Text] [Related]
12. Clinical and metabolic abnormalities in a boy with dietary deficiency of biotin.
Sweetman L; Surh L; Baker H; Peterson RM; Nyhan WL
Pediatrics; 1981 Oct; 68(4):553-8. PubMed ID: 7322688
[TBL] [Abstract][Full Text] [Related]
13. Identification of 3-hydroxy-3-ethylglutaric acid in urine of patients with propionic acidaemia.
Kuhara T; Inoue Y; Shinka T; Matsumoto I; Matsuo M
Biomed Mass Spectrom; 1983 Dec; 10(12):629-32. PubMed ID: 6200156
[TBL] [Abstract][Full Text] [Related]
14. Acetoacetyl CoA thiolase deficiency presenting as ketotic hypoglycemia.
Leonard JV; Middleton B; Seakins JW
Pediatr Res; 1987 Feb; 21(2):211-3. PubMed ID: 2881245
[TBL] [Abstract][Full Text] [Related]
15. A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria.
Bartlett K; Ng H; Leonard JV
Clin Chim Acta; 1980 Jan; 100(2):183-6. PubMed ID: 6766095
[TBL] [Abstract][Full Text] [Related]
16. A defect in l-isoleucine metabolism associated with alpha-methyl-beta-hydroxybutyric and alpha-methylacetoacetic aciduria: quantitative in vivo and in vitro studies.
Gompertz D; Saudubray JM; Charpentier C; Bartlett K; Goodey PA; Draffan GH
Clin Chim Acta; 1974 Dec; 57(3):269-81. PubMed ID: 4434646
[No Abstract] [Full Text] [Related]
17. An inherited disorder of isoleucine catabolism causing accumulation of alpha-methylacetoacetate and alpha-methyl-beta -hydroxybutyrate, and intermittent metabolic acidosis.
Daum RS; Scriver CR; Mamer OA; Delvin E; Lamm P; Goldman H
Pediatr Res; 1973 Mar; 7(3):149-60. PubMed ID: 4690360
[No Abstract] [Full Text] [Related]
18. Capillary gas chromatographic/mass spectrometric analysis of abnormal metabolites in hypoglycin-treated rat urine.
Hine DG; Tanaka K
Biomed Mass Spectrom; 1984 Jul; 11(7):332-9. PubMed ID: 6478044
[TBL] [Abstract][Full Text] [Related]
19. The occurrence of substituted 3-methyl-3-hydroxyglutaric acids in urine in propionic acidaemia and in beta-ketothiolase deficiency.
Pollitt RJ
Biomed Mass Spectrom; 1983 Apr; 10(4):253-7. PubMed ID: 6133567
[TBL] [Abstract][Full Text] [Related]
20. Lymphocyte propionyl-CoA carboxylase is an early and sensitive indicator of biotin deficiency in rats, but urinary excretion of 3-hydroxypropionic acid is not.
Mock DM; Mock NI
J Nutr; 2002 Jul; 132(7):1945-50. PubMed ID: 12097674
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
