Proton NMR spectra of urine from subjects with multiple acyl-CoA dehydrogenase deficiency, caused by defects in either the electron transport flavoprotein or electron transport flavoprotein ubiquinone oxidoreductase, provide a characteristic and possibly diagnostic metabolite profile. The detection of dimethylglycine and sarcosine, intermediates in the oxidative degradation of choline, should discriminate between multiple acyl-CoA dehydrogenase deficiency and related disorders involving fatty acid oxidation. The excretion rates of betaine, dimethylglycine (and sarcosine) in these subjects give an estimate of the minimum rates of both choline oxidation and methyl group release from betaine and reveal that the latter is comparable with the calculated total body methyl requirement in the human infant even when choline intake is very low. Our results provide a new insight into the rates of in vivo methylation in early human development.

Conference Article| November 01 1991 Indirect measurement of betaine - homocysteine methyltransferase activity using 1H nmr spectroscopy SHAMUS BURNS; SHAMUS BURNS 1Medical Unit, The London Hospital Medical College, Whitechapel, London E1 1BB., U.K. Search for other works by this author on: This Site PubMed Google Scholar ANDREW JOHNSON; ANDREW JOHNSON *Department of Clinical Biochemistry, Queen Elizabeth Hospital, London E2 8PS, U.K. Search for other works by this author on: This Site PubMed Google Scholar RICHARD A. ILES RICHARD A. ILES 1Medical Unit, The London Hospital Medical College, Whitechapel, London E1 1BB., U.K. Search for other works by this author on: This Site PubMed Google Scholar Biochem Soc Trans (1991) 19 (4): 402S. https://doi.org/10.1042/bst019402s Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn MailTo Cite Icon Cite Get Permissions Citation SHAMUS BURNS, ANDREW JOHNSON, RICHARD A. ILES; Indirect measurement of betaine - homocysteine methyltransferase activity using 1H nmr spectroscopy. Biochem Soc Trans 1 November 1991; 19 (4): 402S. doi: https://doi.org/10.1042/bst019402s Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu toolbar search search input Search input auto suggest filter your search All ContentAll JournalsBiochemical Society Transactions Search Advanced Search Keywords: BHMT: betaine-homocysteine methyl transferase, DMG: N-dimethylglycine, ETF(-QO): electron transfer flavoprotein (-ubiquinone reductase) This content is only available as a PDF. © 1991 Biochemical Society1991 Article PDF first page preview Close Modal You do not currently have access to this content.