Chronic Wasting Disease - Causative Agent

Causative Agent

The agent responsible for CWD (and other TSEs, such as scrapie and bovine spongiform encephalopathy) is commonly thought to be a prion, an abnormal form of a normal protein, known as prion protein (PrP), most commonly found in the central nervous system (CNS), and is capable of spreading to the peripheral nervous system (PNS), thus infecting meat, or muscle, of deer and elk. The abnormal prion protein infects the host animal by promoting conversion of normal cellular prion protein (PrPC) to the abnormal prion form (PrPCWD). The build-up of PrPCWD in the brain is associated with widespread neurodegeneration. An alternative theory gaining some thus far limited acceptance is that Spiroplasma, a bacterium devoid of a conventional cell wall, may be the causative agent. This does not eliminate prions from playing some role, but speculation has suggested prions may even be a byproduct of Spiroplasma.