RESUMO
A new C26 sterol, 22-trans-27-norcholesta-5,22-dien-3 beta-ol, was found in the urine of a 6-year-old girl, with a clinical diagnosis of congenital adrenal hyperplasia of the salt losing type, accompanied by symptoms of mixed sex anatomy and skin pigmentation. The structure of the sterol was determined by comparison with the synthetic compound. The sterol was also detected in ther serum. This appears to be the first case in which a C26 sterol has occurred in mammalia.
Assuntos
Hiperfunção Adrenocortical/urina , Colestadienóis/urina , Criança , Colestadienóis/sangue , Feminino , Humanos , Noresteroides/sangue , Noresteroides/urinaAssuntos
Androgênios/farmacologia , Lipídeos/sangue , Lipoproteínas/sangue , Androstanos/farmacologia , Androsterona/farmacologia , Animais , Apoproteínas/biossíntese , Transporte Biológico , Proteínas Sanguíneas/metabolismo , Isótopos de Carbono , Cães , Feminino , Humanos , Metabolismo dos Lipídeos , Erros Inatos do Metabolismo Lipídico/metabolismo , Lipase Lipoproteica/sangue , Lipoproteínas/biossíntese , Lipoproteínas HDL/sangue , Lipoproteínas LDL/sangue , Lipoproteínas VLDL/sangue , Masculino , Metiltestosterona/farmacologia , Noresteroides/sangue , Ratos , Fatores Sexuais , Testolactona/farmacologia , Testosterona/farmacologiaRESUMO
Here, we report evidence for the production of ozone in human disease. Signature products unique to cholesterol ozonolysis are present within atherosclerotic tissue at the time of carotid endarterectomy, suggesting that ozone production occurred during lesion development. Furthermore, advanced atherosclerotic plaques generate ozone when the leukocytes within the diseased arteries are activated in vitro. The steroids produced by cholesterol ozonolysis cause effects that are thought to be critical to the pathogenesis of atherosclerosis, including cytotoxicity, lipid-loading in macrophages, and deformation of the apolipoprotein B-100 secondary structure. We propose the trivial designation "atheronals" for this previously unrecognized class of steroids.