Structural highlights
Disease
PRIO_ODOHE Found in high quantity in the brain of animals infected with the degenerative neurological disease known as chronic wasting disease (CWD).
Function
PRIO_ODOHE Its primary physiological function is unclear. Has cytoprotective activity against internal or environmental stresses. 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).[UniProtKB:P04156][UniProtKB:P04925]
See Also