Saposins A, B, C, and D in plasma of patients with lysosomal storage disorders

M. H Y Chang, Colleen A. Bindloss, Gregory A. Grabowski, Xiaoyang Qi, Bryan Winchester, John J. Hopwood, Peter J. Meikle

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Background: Early diagnosis of lysosomal storage disorders (LSDs), before the onset of irreversible pathology, will be critical for maximum efficacy of many current and proposed therapies. To search for potential markers of LSDs, we measured saposins A, B, C, and D in patients with these disorders. Methods: Four time-delayed fluorescence immuno-quantification assays were used to measure each of the saposins in plasma from 111 unaffected individuals and 334 LSD-affected individuals, representing 28 different disorders. Results: Saposin A was increased above the 95th centile of the control population in 59% of LSD patients; saposins B, C, and D were increased in 25%, 61%, and 57%, respectively. Saposins were increased in patients from several LSD groups that in previous studies did not show an increase of lysosome-associated membrane protein-1 (LAMP-1). Conclusion: Saposins may be useful markers for LSDs when used in conjunction with LAMP-1. (C) 2000 American Association for Clinical Chemistry.

Original languageEnglish
Pages (from-to)167-174
Number of pages8
JournalClinical Chemistry
Issue number2
Publication statusPublished - 1 Mar 2000
Externally publishedYes

ASJC Scopus subject areas

  • Clinical Biochemistry
  • Biochemistry, medical

Cite this

Chang, M. H. Y., Bindloss, C. A., Grabowski, G. A., Qi, X., Winchester, B., Hopwood, J. J., & Meikle, P. J. (2000). Saposins A, B, C, and D in plasma of patients with lysosomal storage disorders. Clinical Chemistry, 46(2), 167-174.