Prion topology and toxicity.
Inactivation of mahogunin, an E3 ubiquitin ligase, causes a spongiform encephalopathy resembling prion disease. Chakrabarti and Hegde (2009) now report that prion proteins with aberrant topologies inactivate mahogunin, providing a plausible explanation for certain aspects of prion pathology.
Aguzzi
Adriano
A
Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland. adriano.aguzzi@usz.ch
Steele
Andrew D
AD
D000818:Animals; D006801:Humans; D051379:Mice; D018621:PrPC Proteins / Q000737:chemistry / Q000378:metabolism*; D017096:Prion Diseases / Q000378:metabolism*; D044767:Ubiquitin-Protein Ligases / Q000737:chemistry / Q000378:metabolism
D018621:PrPC Proteins; C470690:MGRN1 protein, human; C470689:Mgrn1 protein, mouse; D044767:Ubiquitin-Protein Ligases