| Identification of galactitol and galactonate in red blood cells by gas chromatography/mass spectrometry. | |
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MedLine Citation:
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PMID: 12104079 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Because the products of alternate pathways of galactose metabolism, galactitol and galactonate are important in galactosemia, we sought to identify these compounds in red blood cells (RBC). METHODS: RBC extracts were trimethylsilylated (TMS) and analyzed by gas chromatography/mass spectrometry (GC/MS). RESULTS: The presence of both galactitol and galactonate was identified in RBC of 15 galactosemic and 13 normal subjects by their mass spectra and chromatographic comparisons with both unlabeled and 13C labeled standards. The levels in RBC of galactosemics appear to be much higher than those of normal subjects. CONCLUSION: The determination of these compounds in RBC along with galactose-1-phosphate (gal-1-P) in the same procedure provides the potential for their use in better monitoring of diet therapy in galactosemic patients. |
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Authors:
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Jie Chen; Claire T Yager; Robert A Reynolds; Stanton Segal |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Clinica chimica acta; international journal of clinical chemistry Volume: 322 ISSN: 0009-8981 ISO Abbreviation: Clin. Chim. Acta Publication Date: 2002 Aug |
Date Detail:
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Created Date: 2002-07-09 Completed Date: 2002-10-11 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 1302422 Medline TA: Clin Chim Acta Country: Netherlands |
Other Details:
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Languages: eng Pagination: 37-41 Citation Subset: IM |
Copyright Information:
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Copyright 2002 Elsevier Science B.V. |
Affiliation:
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Metabolic Research Laboratory, The Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, 402 ARC, Philadelphia, PA 19104, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Erythrocytes
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chemistry* Galactitol / blood* Galactosemias / blood, diet therapy Gas Chromatography-Mass Spectrometry / methods* Humans Sugar Acids / blood* |
| Chemical | |
Reg. No./Substance:
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0/Sugar Acids; 13382-27-9/galactonic acid; 608-66-2/Galactitol |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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