Literature DB >> 5656832

Identification of beta-hydroxyisovaleric acid in the urine of a patient with isovaleric acidemia.

K Tanaka, J C Orr, K J Isselbacher.   

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Year:  1968        PMID： 5656832     DOI： 10.1016/0005-2760(68)90107-0

Source DB:  PubMed          Journal:  Biochim Biophys Acta        ISSN： 0006-3002


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  14 in total

Review 1.  Advances in electronic-nose technologies developed for biomedical applications.

Authors:  Alphus D Wilson; Manuela Baietto
Journal:  Sensors (Basel)       Date:  2011-01-19       Impact factor: 3.576

2.  Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects.

Authors:  K Tanaka; R Mandell; V E Shih
Journal:  J Clin Invest       Date:  1976-07       Impact factor: 14.808

3.  Determination of β-hydroxy-β-methylbutyrate concentration and enrichment in human plasma using chemical ionization gas chromatography tandem mass spectrometry.

Authors:  Dillon K Walker; John J Thaden; Agata Wierzchowska-McNew; Marielle P K J Engelen; Nicolaas E P Deutz
Journal:  J Chromatogr B Analyt Technol Biomed Life Sci       Date:  2016-11-09       Impact factor: 3.205

4.  Inhibition of 3-methylcrotonyl-CoA carboxylase explains the increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients.

Authors:  Paula B M Luís; Jos P Ruiter; Lodewijk IJlst; Luísa Diogo; Paula Garcia; Isabel Tavares de Almeida; Marinus Duran; Ronald J Wanders; Margarida F B Silva
Journal:  J Inherit Metab Dis       Date:  2011-12-22       Impact factor: 4.982

Review 5.  An introduction to gas chromatography-mass spectrometry and the inherited organic acidemias.

Authors:  S I Goodman
Journal:  Am J Hum Genet       Date:  1980-11       Impact factor: 11.025

6.  4-hydroxyisovaleric acid: a new metabolite in isovaleric acidemia.

Authors:  W Lehnert; H Niederhoff
Journal:  Eur J Pediatr       Date:  1981-07       Impact factor: 3.183

7.  Two sisters with isovaleric acidaemia, multiple attacks of ketoacidosis and normal development.

Authors:  M Duran; F J van Sprang; J G Drewes; L Bruinvis; D Ketting; S K Wadman
Journal:  Eur J Pediatr       Date:  1979-06-28       Impact factor: 3.183

8.  Stable isotope dilution analysis of 3-hydroxyisovaleric acid in amniotic fluid: contribution to the prenatal diagnosis of inherited disorders of leucine catabolism.

Authors:  C Jakobs; L Sweetman; W L Nyhan; S Packman
Journal:  J Inherit Metab Dis       Date:  1984       Impact factor: 4.982

Review 9.  "Classical organic acidurias": diagnosis and pathogenesis.

Authors:  Guglielmo Rd Villani; Giovanna Gallo; Emanuela Scolamiero; Francesco Salvatore; Margherita Ruoppolo
Journal:  Clin Exp Med       Date:  2016-09-09       Impact factor: 3.984

10.  Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.

Authors:  W J Rhead; K Tanaka
Journal:  Proc Natl Acad Sci U S A       Date:  1980-01       Impact factor: 11.205
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