The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases. This item is unavailable.The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases

Prions are an entirely new class of pathogens, and scientists are just beginning to understand them. This book tells the strange story of their discovery, and the medical controversies that swirl around them.

THE HUMAN VERSION OF MAD COW DISEASE...

In 1996, British doctors were horrified to discover that mad cow disease (BSE), an affliction that had been plaguing British cattle for ten years, had jumped the species barrier and was appearing in humans as variant Creutzfedlt-Jakob disease (vCJD). Not unlike the mad cows, victims of vCJD suffer from a degenerative neurological disorder that peppers the brain with microscopic holes, causing dementia, loss of motor control, and certain death. What alarms researchers and public health officials worldwide is that the incubation period for vCJD may be as long as 10 or even 15 years, and during this period those infected are symptom-free. And because the disease is so far undetectable except by autopsy, there is no way of knowing with certainty how many people have already been infected. In fact, even travelers who spent time in the U.K. from the early 1980s through the mid-1990s are still considered to be at some risk. What's more, although the U.S. has not detected any mad cows within its borders, there are plenty of mad deer running free in several states, and the disease afflicting them is a BSE-type neurological disorder. Called chronic wasting disease (CWD), the illness in these deer has yet to be linked with any human deaths. But given BSE's ability to jump species, there are no guarantees.

In 'The Pathological Protein', Philip Yam describes how, in this atmosphere of uncertainty, scientists have discovered that the agent of disease in vCJD and a host of other devastating neurological disorders is a bizarre, misshapen version of a protein called a 'prion'. Once introduced into the human neurological system, malformed prions recruit the body's own normal prion proteins, giving them the same pathological ability to destroy brain tissue. Unlike the better-known pathogens that afflict humans - bacteria, viruses, and parasites - prions have so far proved resistant to drug therapies and even standard sterilization. No amount of cooking infected meat will prove effective against them.

In a medical detective story with an undercurrent of urgency, Yam describes how the mysterious prion was discovered, how it has been linked to a number of exotic and poorly understood illneses, and how likely it is that scientists will soon find effective tools for controlling its spread, diagnosing its presence, and treating the devastating disorders it causes.