Ann Lab Med.  2015 Jan;35(1):165-168. 10.3343/alm.2015.35.1.165.

Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry Measurement of Leukocyte Arylsulfatase A Activity Using a Natural Substrate

• Han M
• Jun SH
• Song SH
• Park HD
• Park KU
• Song J

• KMID: 2363170
• DOI: http://doi.org/10.3343/alm.2015.35.1.165

Abstract

No abstract available.

MeSH Terms

Adult
Cerebroside-Sulfatase/*metabolism
Child, Preschool
*Chromatography, High Pressure Liquid
Enzyme Assays/instrumentation/*methods
Female
Humans
Kinetics
Leukocytes/*enzymology
Leukodystrophy, Metachromatic/diagnosis/enzymology
Male
Middle Aged
Reference Standards
Substrate Specificity
Sulfoglycosphingolipids/analysis/metabolism/standards
*Tandem Mass Spectrometry/standards
Cerebroside-Sulfatase
Sulfoglycosphingolipids

Figure

• 

  Fig. 1 Representative ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) multiple reaction monitoring (MRM) chromatograms from the mixture of S, IS and standard (A) and the mixture of ARSA enzyme reactions in the leukocyte samples from normal subjects (B) and metachromatic leukodystrophy patients (C).Abbreviations: ARSA, arylsulfatase A; IS, internal standard; S, substrate; P, product.

• 

  Fig. 2 Comparison of arylsufatase A activities measured by ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) in leukocytes samples from three patients with MLD (all 2-yr-old females) and one patient with pseudodeficiency (PD, a 47-yr-old female), one obligate heterozygote (OH, a 33-yr-old male) and 38 normal adults (females, n=21, age=61±14 yr; males, n=17, age=57±17 yr) (A). Passing-Bablok regression analysis between the UPLC-MS/MS method and the spectrophotometric assay (B).Abbreviations: PD, pseudodeficiency; OH, obligate heterozygote; MLD, metachromatic leukodystrophy.

Reference

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