Anti-Prion protein PrP/PRNP Antibody Picoband® Fluoro647 Conjugated

PRNP (prion protein), also known as CD230 and PRP, is a protein that in humans 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. PRNP is highly conserved through mammals, lending credence to application of conclusions from test animals such as mice. Comparison between primates is especially similar, ranging from 92.9-99.6% similarity in amino acid sequences.

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. Bounhar, Y., Zhang, Y., Goodyer, C. G., LeBlanc, A.Prion protein protects human neurons against Bax-mediated apoptosis.J. Biol. Chem. 276: 39145-39149, 2001. 2. Brown, P., Galvez, S., Goldfarb, L. G., Nieto, A., Cartier, L., Gibbs, C. J., Jr., Gajdusek, D. C.Familial Creutzfeldt-Jakob disease in Chile is associated with the codon 200 mutation of the PRNP amyloid precursor gene on chromosome 20.J. Neurol. Sci. 112: 65-67, 1992. 3. Puckett, C., Concannon, P., Casey, C., Hood, L.Genomic structure of the human prion protein gene.Am. J. Hum. Genet. 49: 320-329, 1991.

Cell membrane; Lipid-anchor, GPI-anchor. Golgi apparatus. Targeted to lipid rafts via association with the heparan sulfate chains of GPC1. Colocates, in the presence of CU (2+), to vesicles in para- and perinuclear regions, where both proteins undergo internalization. Heparin displaces PRNP from lipid rafts and promotes endocytosis.