Prion urine comprises a glycosaminoglycan-light chain IgG complex that can be stained by Congo red.
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.
Halimi
Michele
M
Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital, Jerusalem, Israel.
Dayan-Amouyal
Yael
Y
Kariv-Inbal
Zehavit
Z
Friedman-Levi
Yael
Y
Mayer-Sonnenfeld
Tehila
T
Gabizon
Ruth
R
D002352:Carrier Proteins / Q000187:drug effects / Q000652:urine*; D016022:Case-Control Studies; D004396:Coloring Agents / Q000494:pharmacology*; D003224:Congo Red / Q000494:pharmacology*; D007562:Creutzfeldt-Jakob Syndrome / Q000652:urine*; D006025:Glycosaminoglycans / Q000652:urine*; D006801:Humans; D011328:Prions / Q000737:chemistry / Q000652:urine*; D011485:Protein Binding / Q000187:drug effects; D013048:Specimen Handling
D002352:Carrier Proteins; D004396:Coloring Agents; D006025:Glycosaminoglycans; D011328:Prions; C029702:prolactin-binding protein; D003224:Congo Red