PrionproteinistheproteinresponsIBLeforhumanCreutzfeldt-JakobDisease(CJD),andsomaticmutationsofthepriongeneareresponsibleforGerstmann-Straussler-ScheinkerdiseaseandFatalFamilialInsomnia.ThebovinehomologoftheprionproteinisresponsibleforbovineSpongioformEncephalopathy(BSE),alsoknownas“MadCowDisease.”Inallcases,theprionproteinhastwofoldingstates,withonestatebeingthe“normal”non-pathologicalform,andtheotherstateinducingaspongioformencephalopathy.
Normalprionproteinsproducednaturallyinthebraininteractwiththeamyloid-βpeptidesthatarehallmarksofAlzheimer"sdisease.Blockingthisinteractioninpreparationsmadefrommousebrainshaltedsomeneurologicaldefectscausedbytheaccumulationofamyloid-βpeptide.Itwaspreviouslythoughtthatonlyinfectiousprionproteins,ratherthantheirnormal,non-infectiouscounterparts,playedaroleinbraindegeneration(Nature,doi:10.1038/news.2009.121).
Image:PrionProteinstainingofculturedfibroblastscellstransientlytransfectedwithaplasmidcontainingtheprionproteinCDNA.
SouthernBiotech/Goat Anti-Human Lambda-Alexa Fluor® 555/2070-32/1.0 mg
vectorlabs/Biotinylated Aleuria Aurantia Lectin (AAL)/B-1395/1 mg
vectorlabs/VECTASTAIN® Elite® ABC-HRP Kit (Peroxidase, Mouse IgG)/PK-6102/1 kit
vectorlabs/VECTASTAIN® Elite® ABC-HRP Kit (Peroxidase, Universal)/PK-6200/1 kit
vectorlabs/Unconjugated Musa Paradisiaca (Banana) Lectin (BanLec)/L-1410/5 mg
GiottoBiotech/Calmodulin N60D/5 mg/G02CLM60cn