Clinica Chimica Acta 1978-12-01

Organic acid excretion in a patient with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency: facts and artefacts.

M Duran, D Ketting, S K Wadman, C Jakobs, R B Schutgens, H A Veder

文献索引：Clin. Chim. Acta 90(2) , 187-93, (1978)

摘要

The organic acid excretion in a patient with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency is described. In addition to 3-hydroxy-3-methylglutaric, 3-methylglutaconic, 3-methylglutaric and 3-hydroxyisovaleric acids, substantial amounts of glutaric and adipic acids were repeatedly observed. 3-Methylcrotonic acid and possibly one of its isomers was found to be formed artificially from 3-methylglutaconic acid.

结构式	名称/CAS号	分子式	全部文献
	3,3-二甲基丙烯酸 CAS:541-47-9	C₅H₈O₂

Chemically engineering ligand selectivity at the free fatty ...
2012-12-01
[FASEB J. 26 , 4951-65, (2012)]

Structure-activity relationships of trans-substituted-propen...
2011-05-01
[Bioorg. Med. Chem. Lett. 21 , 2736-9, (2011)]

Tritium-labelled isovaleryl-RYYRIK-NH2 as potential antagoni...
2014-11-01
[Bioorg. Med. Chem. 22(21) , 5902-9, (2014)]

3-methyl-3-butenoic acid: an artefact in the urinary metabol...
1980-09-08
[Clin. Chim. Acta 106(1) , 85-9, (1980)]

Beta-methylcrotonic aciduria associated with lactic acidosis...
1976-02-01
[J. Pediatr. 88(2) , 229-35, (1976)]

Organic acid excretion in a patient with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency: facts and artefacts.

摘要

相关化合物