RESUMO
[Alpha-13C]- and [alpha,beta-13C]valine were administered sequentially to a patient with methylmalonicacidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl-CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by 13C nuclear magnetic resonance spectroscopy. Contrary to the widely accepted view, the results show unequivocally that methylmalonic acid semialdehyde is decarboxylated to propionate before conversion to methylmalonyl CoA.
Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/metabolismo , Malonatos/metabolismo , Ácido Metilmalônico/metabolismo , Propionatos/metabolismo , Valina/metabolismo , Criança , Coenzima A/metabolismo , Humanos , Espectroscopia de Ressonância Magnética , Masculino , Ácido Metilmalônico/urinaAssuntos
Cloro , Colestanos , Oximas , Acetatos , Fenômenos Químicos , Química , Deutério , Estabilidade de Medicamentos , Radicais Livres , Cetosteroides , Espectrometria de Massas , Modelos Químicos , Nitrocompostos , RadioisótoposAssuntos
Sequência de Aminoácidos , Histidina , Peptídeos , Análise Espectral , Espectrometria de Massas , Métodos , MetilaçãoAssuntos
Antineoplásicos/síntese química , Isoquinolinas/síntese química , Piridinas/síntese química , Tiossemicarbazonas/síntese química , Animais , Antineoplásicos/uso terapêutico , Isoquinolinas/uso terapêutico , Leucemia L1210/tratamento farmacológico , Espectroscopia de Ressonância Magnética , Metilação , Camundongos , Camundongos Endogâmicos , Piridinas/uso terapêutico , Relação Estrutura-Atividade , Tiossemicarbazonas/uso terapêuticoRESUMO
A sample of lunar material from Apollo 11 was subjected to analysis by several techniques, which included mass spectrometry, gas chromatography, liquid chromatography, and nuclear magnetic resonance and their variations, in an effort to detect the presence of organic compounds. None were found. On the basis of the sensitivity ascribed to certain of the methods employed, it is assumed that if organic matter were present it would exist in concentrations less than 1 part per million. phere.
