4hmr
From Proteopedia
Contents |
Crystal structure of mutant rabbit PRP 121-230 (S170N/S174N)
Template:ABSTRACT PUBMED 23675452
Disease
[PRIO_RABIT] Note=Found in high quantity in the brain of humans and animals infected with degenerative neurological diseases such as kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler syndrome (GSS), scrapie, bovine spongiform encephalopathy (BSE), transmissible mink encephalopathy (TME), etc.
Function
[PRIO_RABIT] May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May play a role in iron uptake and iron homeostasis. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro). Association with GPC1 (via its heparan sulfate chains) targets PRNP to lipid rafts. Also provides Cu(2+) or ZN(2+) for the ascorbate-mediated GPC1 deaminase degradation of its heparan sulfate side chains (By similarity).
About this Structure
4hmr is a 2 chain structure with sequence from Oryctolagus cuniculus. Full crystallographic information is available from OCA.
Reference
- Sweeting B, Brown E, Khan MQ, Chakrabartty A, Pai EF. N-Terminal Helix-Cap in alpha-Helix 2 Modulates beta-State Misfolding in Rabbit and Hamster Prion Proteins. PLoS One. 2013 May 10;8(5):e63047. doi: 10.1371/journal.pone.0063047. Print 2013. PMID:23675452 doi:10.1371/journal.pone.0063047
