Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu.

Title:

Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu.

identifier:	1314181
description:	epitope description:E219 antigen name:Major prion protein host organism:Mus musculus BALB/c antibody name:#41, #71
aggregation:	instance of dataset
availability:	available
primaryPublications:	10889337

study type:	b cell assays
subject species:	Homo sapiens

fullName:	T Muramoto N Kitamoto C Sano Y Hayashi C Yutani T Kitamoto

method:	immuno staining

name:	Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu.
description:	This residue was determined to be a critical residue for antibody recognition. The antibodies, which recognized human PrP with 219Glu but not that with 219Lys, were utilized to determine the allelic origin of abnormal PrP deposited in the brain of a patient with Gerstmann-Straussler syndrome (GSS) with 102Leu/219Lys encoded by the same allele. Abnormal PrP was exclusively of mutant allelic origin, suggesting that 219Lys may be permissive to the formation of abnormal PrP in GSS. Similar results were obtained in Western blots.

name:	iedb
homePage:	http://www.iedb.org

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