Rabbit anti-Human Prion Protein Polyclonal Antibody

Uniprot Id

P04156

Target Species

Human

Target Name

PRNP

Target Full Name

Major prion protein

Target 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.

Target Involvement

Creutzfeldt-Jakob disease (CJD); Fatal familial insomnia (FFI); Gerstmann-Straussler disease (GSD); Huntington disease-like 1 (HDL1); Kuru (KURU); Spongiform encephalopathy with neuropsychiatric features (SENF)

Target Subcellular Location

Cell membrane; Lipid-anchor, GPI-anchor. Golgi apparatus.

Target Protein Families

Prion family

Target Synonyms

CJD; GSS; PrP; ASCR; KURU; PRIP; PrPc; CD230; AltPrP; p27-30; PrP27-30; PrP33-35C; Prion Protein

Target Background

The protein encoded by this gene is a membrane glycosylphosphatidylinositol-anchored glycoprotein that tends to aggregate into rod-like structures. The encoded protein contains a highly unstable region of five tandem octapeptide repeats. This gene is found on chromosome 20, approximately 20 kbp upstream of a gene which encodes a biochemically and structurally similar protein to the one encoded by this gene. Mutations in the repeat region as well as elsewhere in this gene have been associated with Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Straussler disease, Huntington disease-like 1, and kuru. An overlapping open reading frame has been found for this gene that encodes a smaller, structurally unrelated protein, AltPrp. Alternative splicing results in multiple transcript variants.