2joh

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Template:STRUCTURE 2joh

Contents

NMR structure of rabbit prion protein mutation S173N

Template:ABSTRACT PUBMED 20639199

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

2joh is a 1 chain structure with sequence from Oryctolagus cuniculus. Full experimental information is available from OCA.

See Also

Reference

  • Wen Y, Li J, Yao W, Xiong M, Hong J, Peng Y, Xiao G, Lin D. Unique structural characteristics of the rabbit prion protein. J Biol Chem. 2010 Jul 16. PMID:20639199 doi:10.1074/jbc.M110.118844
  • Li J, Mei FH, Xiao GF, Guo CY, Lin DH. 1H, 13C and 15N resonance assignments of rabbit prion protein (91-228). J Biomol NMR. 2007 Jun;38(2):181. Epub 2007 Apr 6. PMID:17415669 doi:10.1007/s10858-006-9115-9

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