TY - JOUR
T1 - Urine organic acid analysis for inherited metabolic disease using gas chromatography-mass spectrometry.
AU - Jones, Patricia M.
AU - Bennett, Michael J.
PY - 2010
Y1 - 2010
N2 - Urine organic acid analysis is an essential component of the workup of the patient suspected to have an inborn error of metabolism (IEM). Urine contains several hundred different organic acids, which arise from a multitude of different sources including both normal and abnormal metabolism. They may also arise from drugs and drug metabolism or from xenobiotics and dietary supplements. In addition to the diagnosis of inborn errors of metabolism, the identification of organic acids in a urine sample has a wide range of potential applications, including toxicology and poisonings. The method described below extracts the acidic fraction from urine samples, derivatizes the extracted compounds, and identifies intermediate metabolites by GC-MS. The method utilizes electron impact ionization gas chromatography-mass spectrometry (GC-MS) with total ion collection.
AB - Urine organic acid analysis is an essential component of the workup of the patient suspected to have an inborn error of metabolism (IEM). Urine contains several hundred different organic acids, which arise from a multitude of different sources including both normal and abnormal metabolism. They may also arise from drugs and drug metabolism or from xenobiotics and dietary supplements. In addition to the diagnosis of inborn errors of metabolism, the identification of organic acids in a urine sample has a wide range of potential applications, including toxicology and poisonings. The method described below extracts the acidic fraction from urine samples, derivatizes the extracted compounds, and identifies intermediate metabolites by GC-MS. The method utilizes electron impact ionization gas chromatography-mass spectrometry (GC-MS) with total ion collection.
UR - http://www.scopus.com/inward/record.url?scp=77950358809&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77950358809&partnerID=8YFLogxK
U2 - 10.1007/978-1-60761-459-3_41
DO - 10.1007/978-1-60761-459-3_41
M3 - Article
C2 - 20077094
AN - SCOPUS:77950358809
SN - 1064-3745
VL - 603
SP - 423
EP - 431
JO - Methods in molecular biology (Clifton, N.J.)
JF - Methods in molecular biology (Clifton, N.J.)
ER -