Physical Sciences and Mathematics Commons^™

Profiling Sterols In Cerebrotendinous Xanthomatosis: Utility Of Girard Derivatization And High Resolution Exact Mass Lc-Esi-Msn Analysis, Andrea E. Debarber, Yana Sandlers, Anuradha S. Pappu, Louise S. Merkens, P. Barton Duell, Steven R. Lear, Sandra K. Erickson, Robert D. Steiner

Chemistry Faculty Publications

In this study we profile free 3-oxo sterols present in plasma from patients affected with the neurodegenerative disorder of sterol and bile acid metabolism cerebrotendinous xanthomatosis (CTX), utilizing a combination of charge-tagging and LC-ESI-MSn performed with an LTQ-Orbitrap Discovery instrument. In addition, we profile sterols in plasma from 24-month-old cyp27A1 gene knockout mice lacking the enzyme defective in CTX. Charge-tagging was accomplished by reaction with cationic Girard's P (GP) reagent 1-(carboxymethyl) pyridinium chloride hydrazide, an approach uniquely suited to studying the 3-oxo sterols that accumulate in CTX, as Girard's reagent reacts with the sterol oxo moiety to form charged hydrazone …