MISFOLDING PROTEIN DISEASE SUMMARY Prion structure and function

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MISFOLDING PROTEIN &DISEASE SUMMARY ü Prion : structure and function ü Prion 的致病機制: Pr.

MISFOLDING PROTEIN &DISEASE SUMMARY ü Prion : structure and function ü Prion 的致病機制: Pr. PC→Pr. PSC→Disease ü Misfolding protein 的修正 ü Disease: Kuru, CJD, Parkinson, AD ü 疾病的檢查

Prion : structure and function

Prion : structure and function

C Pr. P SC Pr. P

C Pr. P SC Pr. P

Misfolding protein 的修正

Misfolding protein 的修正

正常人體該如何排解 misfolded protein?

正常人體該如何排解 misfolded protein?

Disease

Disease

Distribution of infectivity in the human body Infectivity category Tissues, secretions, and excretions High

Distribution of infectivity in the human body Infectivity category Tissues, secretions, and excretions High infectivity Brain, spinal cord, eye Low infectivity CSF, kidney, liver, lung, lymph node/spleen, placenta No detectable infectivity Adipose tissue, adrenal gland, gingival tissue, heart muscle, intestine, peripheral nerve, prostate, skeletal muscle, testis, thyroid, tears, nasal mucous, saliva, sweat, serous exudate, milk, semen, urine, feces, blood Data based on primates inoculated with CJD tissues and naturally occurring animal TSEs (WHO, 2000)

KURU

KURU

Histological features of TSE kuru Scrapie v. CJD BSE

Histological features of TSE kuru Scrapie v. CJD BSE

Degenerative diseases n n Parkinson Disease 巴金 森症 Huntington’s Disease 漢丁頓式病 Amyotrophic Lateral Sclerosis

Degenerative diseases n n Parkinson Disease 巴金 森症 Huntington’s Disease 漢丁頓式病 Amyotrophic Lateral Sclerosis (ALS) 肌萎縮 側索硬化症 Alzheimer Disease (AD) 阿滋海默症 Huntington’s disease (caudate nucleus and frontal cortex)

Alzheimer disease 不正常 正常

Alzheimer disease 不正常 正常

Parkinson Disease Gross Depigmentation of the substantia nigra in PD (left). Normal sustantia nigra

Parkinson Disease Gross Depigmentation of the substantia nigra in PD (left). Normal sustantia nigra (right)

腦電波出現週期性棘波(periodic sharp wave pattern) 70%會出現此症狀

腦電波出現週期性棘波(periodic sharp wave pattern) 70%會出現此症狀

磁共振成像 枕核丘腦(the pulvinar nuclei of the thalamus) 出現對稱性的高信號

磁共振成像 枕核丘腦(the pulvinar nuclei of the thalamus) 出現對稱性的高信號