| Detection by mass spectrometry of highly increased amount of S-sulfonated transthyretin in serum from a patient with molybdenum cofactor deficiency. | |
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MedLine Citation:
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PMID: 10759156 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Serum transthyretin has several isoforms, most of which are caused by disulfide linkage with cysteine residue at position 10. We found an ion peak 80 D larger than unmodified transthyretin by electrospray ionization mass spectrometry and assigned it to S-sulfonated transthyretin. The peak height was <2% of total transthyretin in control sera from more than 200 individuals including infants. Transthyretin from a patient with molybdenum cofactor deficiency was analyzed, and the peak was prominent, higher than 85% of total transthyretin. In patients with this disease, the presence of elevated levels of sulfite leads to the formation of S-sulfonated cysteine. The peak can be used as a diagnostic marker for molybdenum cofactor deficiency, although more sera from patients with this disease should be tested. |
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Authors:
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M Kishikawa; T Nakanishi; A Shimizu; M Yoshino |
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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: Pediatric research Volume: 47 ISSN: 0031-3998 ISO Abbreviation: Pediatr. Res. Publication Date: 2000 Apr |
Date Detail:
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Created Date: 2000-06-01 Completed Date: 2000-06-01 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0100714 Medline TA: Pediatr Res Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 492-4 Citation Subset: IM |
Affiliation:
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Department of Clinical Pathology, Osaka Medical College, Takatsuki, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Child, Preschool Coenzymes* Humans Infant Male Metabolism, Inborn Errors / blood* Metalloproteins / metabolism* Molybdenum / metabolism* Prealbumin / chemistry, metabolism* Pteridines / metabolism* Spectrometry, Mass, Secondary Ion / methods* Sulfonic Acids / chemistry |
| Chemical | |
Reg. No./Substance:
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0/Coenzymes; 0/Metalloproteins; 0/Prealbumin; 0/Pteridines; 0/Sulfonic Acids; 73508-07-3/molybdenum cofactor; 7439-98-7/Molybdenum |
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