Prion urine comprises a glycosaminoglycan-light chain IgG complex that can be stained by Congo red

Michele Halimi, Yael Dayan-Amouyal, Zehavit Kariv-Inbal, Yael Friedman-Levi, Tehila Mayer-Sonnenfeld, Ruth Gabizon*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

Light chain IgG, a known amyloidotic protein, is present in the urine of prion disease affected individuals in a protease resistant form. In addition, it was shown recently that prion urine samples comprise a significant excess of glycosaminoglycans. Since amyloidotic proteins and glycosaminoglycans are the major components of amyloid aggregates, a Congo red dot blot assay was developed for detection of Creutzfeldt-Jacob disease (CJD) in urine. This assay was also positive for about 10% of patients suffering from diseases such as Alzheimer disease, cerebrovascular attacks and multiple sclerosis, but negative for healthy controls. Both glycosaminoglycans and proteins such as light chain IgG were required for the binding of Congo red to the urine fractions, as shown by the fact that Proteinase K digestion of the samples either after guanidine or after choindrotinase abolished the Congo red signal from the CJD samples.

Original languageAmerican English
Pages (from-to)205-210
Number of pages6
JournalJournal of Virological Methods
Volume133
Issue number2
DOIs
StatePublished - May 2006
Externally publishedYes

Keywords

  • CJD
  • Congo red
  • Glycosaminoglycans
  • Light chain IgG
  • Prion
  • Urine

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