France Halts Prion Research
Prions are lethal. They migrate, mutate and kill with unparalleled efficiency. As such prion disease is the fastest-growing cause of death on the planet. It kills most, if not all, mammals.
Unfortunately, prion contamination is spreading around the world via numerous pathways, but scientists and governments are more interested in studying them than containing them. Such questionable priorities recently cost two scientists their lives.
At least two scientists in France contracted a rare and fatal prion brain disease after working in labs that handle prions. As such, France issued a temporary moratorium on all research into prion diseases by public research laboratories for at least three months.
The moratorium was issued after a retired National Research Institute for Agriculture, Food and Environment (INRAE) lab worker who handled prions in the lab developed Creutzfeldt-Jakob disease (CJD).
While the origin of the disease is not certain, this retired lab worker could be the second known case of someone catching a prion disease in a lab run by INRAE. In June 2019, Émilie Jaumain died at age 33 after being infected with prions in 2010. Jaumain pricked her thumb during an experiment with prion-infected mice. Unfortunately, she was wearing latex gloves instead of pierce-proof gloves.
In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD.
Her family is suing the INRAE for manslaughter and endangering life. INRAE finally admitted the likely link between her death and the lab accident. Unfortunately, Jaumain likely exposed friends, family and caregivers to the prion pathogen. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings.
Prions (PREE-ons) are a deadly and unstoppable form of protein that migrates, mutates, multiplies and kills with unparalleled efficiency. Prions were first discovered in the late 1980s as a protein-containing biological agent that replicates itself in living cells without nucleic acid.
Prions cause fatal neurodegenerative disease in humans and other mammals by converting the cellular version of prion protein into a toxic form that erodes the brain and body. Prion disease often is described as a wasting disease that causes a loss of body mass and brain mass.
Prion disease is a spectrum disease. It is clinically known as transmissible spongiform encephalopathy (TSE). As the name suggests, TSEs are transmissible. Deadly prions spread through the bodily fluids and tissue of those carrying prion disease (milk, blood, saliva, mucus, urine, feces, tissue and skin). Prions shed from infected humans are highly transmissible to humans and other mammals.
Alzheimer’s disease, Parkinson’s disease and Creutzfeldt-Jakob disease (CJD) are forms of prion disease. In livestock, prion disease is called mad cow disease. In wildlife, it’s often referred to as chronic wasting disease.
Until recently, few have considered the possibility that Alzheimer’s disease is a transmissible disease. This denial and misinformation has been fueling a public health crisis around the world for years.
Dr. Stanley Prusiner, an American neuroscientist from the University of California at San Francisco, earned a Nobel Prize in 1997 for discovering and characterizing prions and prion disease. President Obama awarded Prusiner the National Medal of Science in 2010 to recognize the importance of his research. Important reforms to policies to protect public health, however, have been elusive. Prusiner’s most recent study confirms that Alzheimer’s disease is a prion disease–also known as transmissible spongiform encephalopathy (TSEs). Once again, it demands reform on many levels to protect public health.
Learn more about Dr. Stanley Prusiner and the Nobel Prize for prion science and prion disease.
