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Allied Journal of Medical Research
|
Volume 2
Page 51
allied
academies
CANCER THERAPY AND ONCOLOGY
NEUROLOGY AND BRAIN DISORDERS
&
International Conference on
International Conference on
J u n e 2 1 - 2 2 , 2 0 1 8 | O s a k a , J a p a n
Joint Event on
SKIN PRION AND ITS IMPLICATIONS IN PRION DISEASES AND
OTHER NEURODEGENERATIVE DISEASES
Zou WQ
Case Western Reserve University, USA
P
rions (or PrP
Sc
) are associated with a group of fatal transmissible prion diseases including sporadic Creutzfeldt-Jakob
disease (sCJD, the most common human prion disease) in humans as well as scrapie, mad cow disease, and chronic
wasting disease in animals. The currently incurable sCJD is transmissible, due to the contamination of abundant infectious
prions in the brain through medical or surgical procedures. Some epidemiological studies have also associated sCJD risk
with non-neurosurgeries, suggesting that prions may be present in other tissues such as skin. In addition, once disease
onset has occurred, the brain becomes inevitably damaged. So, preclinical detection is key to providing the critical window
for early treatments before irreversible brain damages occur once cures become available. Our recent study using the highly
sensitive real-time quaking-induced conversion (RT-QuIC) assay and humanized transgenic (Tg) mice-based bioassay
revealed that the skin of sCJD patients harbors infectious prions (Orrú et al., 2017). Moreover, our new preliminary results
further indicate that skin PrPSc is detectable by RT-QuIC and serial protein misfolding cyclic amplification assays far ahead
of neuropathological changes in prion-infected animal models. Our findings not only raise concerns about the potential
for iatrogenic sCJD transmission via skin but also provide a basis for establishing alternative premortem and postmortem
diagnostic assays for prion diseases. Moreover, they may improve our understanding of the role of other skin misfolded
proteins in the diagnosis and pathogenesis of neurodegenerative diseases such as Alzheimer’s and Parkinson’s diseases
in which disease-specific misfolded proteins have been detected in the skin of patients with these diseases. [Supported by
the CJD Foundation, NIH (NS062787, NS087588 and NS096626), and CDC].
wxz6@case.eduAllied J Med Res 2018, Volume 2