Abnormal Proteins Discovered in Skin of Patients With Rare
Brain Disease
By Denise Grady
NY Times Nov. 22, 2017 NY Times Link.
Finding so-called prions in skin samples may lead to early
diagnosis of Creutzfeldt-Jakob disease. But the discovery also hints at
possible risks from surgery.
The
discovery suggests that skin samples might be used to improve detection of the
disorder, Creutzfeldt-Jakob disease, which now is usually diagnosed with much
more difficult procedures, like brain biopsies or autopsies.
The
researchers said that although the disease had been transmitted decades ago by
corneal transplants and certain neurosurgical procedures, there was no
definitive evidence that other types of surgery had ever spread it. And the
levels found in skin are far lower than those in the brain.
Creutzfeldt-Jakob is the most common member of a family of
disorders that affect people and animals called transmissible spongiform
encephalopathies. The diseases eat holes in the brain, leaving it looking like
a sponge (hence the name). The other human ailments include kuru, fatal
familial insomnia and Gerstmann-Straussler-Scheinker disease.
Principal Investigator, Wen-Quan Zou, M.D.,
PHD, CJD Foundation.
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| Prions from BSE in green (from NY Times article) |
