Quantitative gas chromatography and single-ion detection of aliphatic α-keto acids from urine as their o-trimethylsilylquinoxalinol derivatives

Langenbeck, U.; Hoinowski, A.; Mantel, K.; Möhring, H.

doi:10.1016/s0378-4347(00)81325-0

Cited by 60 publications

(10 citation statements)

References 34 publications

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“…Analysis of the BCKA's in the serum, cerebrospinal fluid and dialysis fluid was done by gas chromatography with nitrogen selective detection, according to Langenbeck et al [14].…”

Section: Methodsmentioning

confidence: 99%

Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

et al. 1980

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“…Analysis of the BCKA's in the serum, cerebrospinal fluid and dialysis fluid was done by gas chromatography with nitrogen selective detection, according to Langenbeck et al [14].…”

Section: Methodsmentioning

confidence: 99%

Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

et al. 1980

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“…Urinary 2-oxoacids were determined by gas chromatography (GC) of the respective O-TMS quinoxalinols [17]. Mass spectrometry (MS) was carried out on a Finnigan model 4000 GC-MS combination [18].…”

Section: Identification and Quantitation Of Metabolites In Body Fluidsmentioning

confidence: 99%

A familial progressive neurodegenerative disease with 2-oxoglutaric aciduria

et al. 1982

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A boy and a girl born to a consanguineous Tunisian couple are suffering from a slowly progressive nervous disorder. Initially they both had normal psychomotor development with acquisition of gait and speech. First symptoms in the boy were athetoid movements during the second year of life. He later lost all motor and language skills and developed muscular rigidity and intention tremor. At the age of five years, he was completely bedridden while he appeared mentally much less affected. His younger sister followed a similar course. The major specific abnormality detected was a strikingly elevated excretion of 2-oxoglutaric acid, which was identified by gas liquid chromatography, mass spectrometry, and enzymatic analysis. 2-oxoglutarate dehydrogenase activity in homogenates of cultured skin fibroblasts was reduced to about 25% of control values in both children. Although the pathogenetic mechanisms leading to brain damage remain obscure, the finding strongly suggest an autosomal recessive neurometabolic disease with predominant involvement of the extrapyramidal system.

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“…The phenolic hydroxyl group is usually further derivatized to the TMS ether to improve the Chromatographie performance of the quinozalinol derivative [542]. Many of the reactions can be performed in aqueous solution and the derivatives determined selectively using a thermionic detector in the case of the amino alcohols.…”

Section: Rchohmentioning

confidence: 99%

Sample Preparation for Chromatographic Analysis

Poole

1991

Chromatography Today

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Quantitative gas chromatography and single-ion detection of aliphatic α-keto acids from urine as their o-trimethylsilylquinoxalinol derivatives

Cited by 60 publications

References 34 publications

Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

A familial progressive neurodegenerative disease with 2-oxoglutaric aciduria

Sample Preparation for Chromatographic Analysis

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