2lh8

From Proteopedia

proteopedia linkproteopedia link

Template:STRUCTURE 2lh8

Contents 1 Syrian hamster prion protein with thiamine 2 Disease 3 Function 4 About this Structure 5 See Also 6 Reference

Syrian hamster prion protein with thiamine

Template:ABSTRACT PUBMED 21848803

Disease

[PRIO_MESAU] 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_MESAU] 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).^[1]

About this Structure

2lh8 is a 1 chain structure with sequence from Mesocricetus auratus. Full experimental information is available from OCA.

See Also

• Prion

Reference

• Perez-Pineiro R, Bjorndahl TC, Berjanskii MV, Hau D, Li L, Huang A, Lee R, Gibbs E, Ladner C, Dong YW, Abera A, Cashman NR, Wishart DS. The Prion Protein Binds Thiamine. FEBS J. 2011 Aug 16. doi: 10.1111/j.1742-4658.2011.08304.x. PMID:21848803 doi:10.1111/j.1742-4658.2011.08304.x

1. ↑ Juanes ME, Elvira G, Garcia-Grande A, Calero M, Gasset M. Biosynthesis of prion protein nucleocytoplasmic isoforms by alternative initiation of translation. J Biol Chem. 2009 Jan 30;284(5):2787-94. doi: 10.1074/jbc.M804051200. Epub 2008, Dec 5. PMID:19059915 doi:10.1074/jbc.M804051200