Anti-Prion protein (PrP)

0.5mg/ml if reconstituted with 0.2ml sterile DI water. Prion protein, also known as CD230 and PRP, is encoded by the PRNP gene. The major prion protein is expressed in the brain and several other tissues. Expression is most predominant in the nervous system but occurs in many other tissues throughout the body. Puckett et al.(1991) identified a RFLP with a high degree of heterozygosity in the 5-prime region of the PRNP gene, which might serve as a useful marker for the pter-p12 region of chromosome 20. PRNP is associated with a variety of cognitive deficiencies and neurodegenerative diseases such as Creutzfeldt-Jakob disease, bovine spongiform encephalopathy, and kuru. Protein function: Its primary physiological function is unclear. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May promote myelin homeostasis through acting as an agonist for ADGRG6 receptor. 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 (PubMed:12732622, PubMed:16492732, PubMed:19242475, PubMed:19568430). [The UniProt Consortium]