Related topics: neurodegenerative diseases

A prion-related protein senses warmer temperature in plants

Many plants and trees flower in the spring when it gets warmer. How plants sense Scientists from the UK, France, Korea and Germany focused on a protein called EARLY FLOWERING3 (ELF3). ELF3 is a key part of the circadian clock ...

Unraveling one of prion disease's deadly secrets

A molecular biologist at the University of Massachusetts Amherst who has for decades studied the nightmarish group of fatal diseases caused by prions—chronic wasting disease in deer, mad cow in cattle and its human analog—credits ...

Researchers say may have found cause of mad cow disease

Researchers said Wednesday they believe they may have found the cause of mad cow disease, while stressing the need to maintain precautionary measures to avoid a potential re-emergence of the illness.

Protein chains that self-form into helical braids

A team of researchers from Durham University in the U.K. and Shaanxi Normal University in China has discovered a type of protein that forms naturally into two main types of helical braids. In their paper published in the ...

Altering pH bumps prions out of danger zone

Prion diseases are scary, incurable and fatal. They first gained notoriety when cows became infected by prion proteins and, in turn, infected people. Fervor surrounding mad cow disease resulted in the U.S. banning imports ...

Novel amyloid structure could lead to new types of antibiotics

The highly pathogenic Staphylococcus aureus bacteria is one of the five most common causes of hospital-acquired infections. In the US alone, approximately 500,000 patients at hospitals contract a staph infection. It is the ...

A prion-like protein discovered in bacteria

(Phys.org)—A pair of researchers at Harvard Medical School has found an instance of a bacterial protein that behaves like a prion when inserted into another type of bacteria. In their paper published in the journal Science, ...

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Bovine spongiform encephalopathy

Bovine spongiform encephalopathy (BSE), commonly known as mad-cow disease (MCD), is a fatal, neurodegenerative disease in cattle, that causes a spongy degeneration in the brain and spinal cord. BSE has a long incubation period, about 4 years, usually affecting adult cattle at a peak age onset of four to five years, all breeds being equally susceptible. In the United Kingdom, the country worst affected, more than 179,000 cattle have been infected and 4.4 million slaughtered during the eradication programme.

It is believed by most scientists that the disease may be transmitted to human beings who eat the brain or spinal cord of infected carcasses. In humans, it is known as new variant Creutzfeldt-Jakob disease (vCJD or nvCJD), and by February 2009, it had killed 164 people in Britain, and 42 elsewhere with the number expected to rise because of the disease's long incubation period. Between 460,000 and 482,000 BSE-infected animals had entered the human food chain before controls on high-risk offal were introduced in 1989.

A British inquiry into BSE concluded that the epidemic was caused by cattle, who are normally herbivores, being fed the remains of other cattle in the form of meat and bone meal (MBM), which caused the infectious agent to spread. The origin of the disease itself remains unknown. The infectious agent is distinctive for the high temperatures at which it remains viable; this contributed to the spread of the disease in Britain, which had reduced the temperatures used during its rendering process. Another contributory factor was the feeding of infected protein supplements to very young calves.

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