Document Detail

Detection by mass spectrometry of highly increased amount of S-sulfonated transthyretin in serum from a patient with molybdenum cofactor deficiency.
MedLine Citation:
PMID:  10759156     Owner:  NLM     Status:  MEDLINE    
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.
M Kishikawa; T Nakanishi; A Shimizu; M Yoshino
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric research     Volume:  47     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  2000 Apr 
Date Detail:
Created Date:  2000-06-01     Completed Date:  2000-06-01     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  492-4     Citation Subset:  IM    
Department of Clinical Pathology, Osaka Medical College, Takatsuki, Japan.
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MeSH Terms
Child, Preschool
Metabolism, Inborn Errors / blood*
Metalloproteins / metabolism*
Molybdenum / metabolism*
Prealbumin / chemistry,  metabolism*
Pteridines / metabolism*
Spectrometry, Mass, Secondary Ion / methods*
Sulfonic Acids / chemistry
Reg. No./Substance:
0/Coenzymes; 0/Metalloproteins; 0/Prealbumin; 0/Pteridines; 0/Sulfonic Acids; 73508-07-3/molybdenum cofactor; 7439-98-7/Molybdenum

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