The human form of the disease is called variant Creutzfeld-Jacobs Disease, or vCJD.
The full form of BSE is Bombay Stock Exchange Or (and sadly more common) Bovine spongiform encephalopathy better known as "Mad Cow Disease" a degenerative nurological condition caused by prions.
Bovine spongiform encephalopathy (BSE), commonly known as "mad cow disease," is a neurodegenerative disease that affects cattle. It is caused by abnormal proteins called prions, which lead to the degeneration of brain tissue, resulting in spongy lesions. BSE can be transmitted to humans through the consumption of infected beef products, leading to a variant form of Creutzfeldt-Jakob disease. The disease has significant implications for public health and cattle farming practices.
Feline spongiform encephalopathy a disease caused by prions in cats.
Chaperone proteins assist in the proper folding of other proteins and help prevent misfolding and aggregation. In the case of bovine spongiform encephalopathy (BSE), a prion disease, the abnormal prion protein (PrP^Sc) can induce misfolding of the normal prion protein (PrP^C) into the pathogenic form. Chaperones may be involved in the misfolding process or in the cellular response to the accumulation of these misfolded proteins, potentially influencing the progression of the disease. Thus, their role can be crucial in understanding the mechanisms of BSE and its effects on neurological function.
Spongiform can be broken down into sponge and form, so that means it has a sponge like quality. In terms of spongiform encephalopathy it means that the brain has the sponge-like quality of being full of small holes.
A prion is a misfolded protein that is considered an infectious agent because they cause properly folded proteins to convert into the misfolded, prion form. In humans, prions cause Creutzfeldt-Jakob disease. In cattle, prions cause mad-cow disease.
Human spongiform encephalitis
Bo-vine Spon-gi-form En-ceph-a-li-tis
There are no known cures for MCD in its human form, which is otherwise known as variant Cruetzfeldt-Jakob Disease (vCJD). Nor are there any cures for MCD in the bovine version, which is otherwise known as Bovine Spongiform Encephalopathy (BSE). As a matter of fact, no form of this disease in any animal that is likely to be affected (sheep, bison, buffalo, elk, deer, etc.) has any sort of cure whatsoever. This disease in sheep and goats is called Scrapie. In cervids, it is called Chronic Wasting Disease. BSE, Scrapie, and CWD are all collectively known as Transmissble Spongiform Encephalopathy (TSE). Thus, TSE is not curable. "There is currently no proven treatment for the underlying process" which causes variant CJD according to the Edinburgh CJD unit in Scotland, in the United Kingdom (July 2006). This is also true for the bovine variant, sheep variant or cervid variant of this disease. The only possible treatment available (if you can call it that) is humane euthanasia of the affected animal.
There are two forms of bovine spongiform encephalopathy (BSE, the scientific name for mad cow disease): infectious and spontaneous. The infectious form is what caused the problems in the 1990s, and this form was often transmitted via the feed. The spontaneous form, which is the only form the world is currently seeing due to a ruminant-to-ruminant feed ban, occurs randomly in older cattle.
BSE is an interesting disease from a medical standpoint because of the causative agent, prions (pronounced pree-ons). A prion is an infectious protein that is similar to a virus, but not a typical virus. Unlike viruses, prions aren't alive, so they can't be killed with the usual disinfectants. The body does not mount a typical viral immune attack against prions, either. Prion proteins can be denatured, but only at extremely high temperatures or with very strong chemicals, either way, not consistent with animal life so these are not treatment options. Diseases caused by prions are referred to as TSEs -- Transmissible Spongiform Encephalopathies.
There are two types of BSE (bovine spongiform encephalopathy, the scientific name for mad cow disease). The first is what the general media picks up on when BSE is mentioned - the infectious prion identified in the 1980s and 1990s that is linked to variant Creutzfeldt-Jakobs Disease in humans. However, this form has been almost entirely eradicated in the world. The second is what the last three cases of BSE in the United States have been - atypical or spontaneous BSE. This is where the normal protein in the brain misfolds on its own and causes a chain reaction.