3nve

From Proteopedia

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Template:STRUCTURE 3nve

Contents 1 MMHFGN segment 138-143 from Syrian Hamster prion 2 Disease 3 Function 4 About this Structure 5 See Also 6 Reference

MMHFGN segment 138-143 from Syrian Hamster prion

Template:ABSTRACT PUBMED 21323366

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

3nve is a 2 chain structure. Full crystallographic information is available from OCA.

See Also

• Prion

Reference

• Apostol MI, Wiltzius JJ, Sawaya MR, Cascio D, Eisenberg D. Atomic structures suggest determinants of transmission barriers in mammalian prion disease. Biochemistry. 2011 Feb 16. PMID:21323366 doi:10.1021/bi101803k

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