著者
徳田 栄一 Stefan L. Marklund 古川 良明
出版者
公益社団法人 日本薬学会
雑誌
YAKUGAKU ZASSHI (ISSN:00316903)
巻号頁・発行日
vol.139, no.7, pp.1015-1019, 2019-07-01 (Released:2019-07-01)
参考文献数
16
被引用文献数
2 2

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease that is characterized by the loss of motor neurons, which results in progressive muscle atrophy. The pathology spreads from the initial site of onset to contiguous anatomic regions. Mutations in the gene encoding Cu/Zn-superoxide dismutase (SOD1) have been identified in a dominantly inherited form of ALS (ALS-SOD1). A major hallmark of ALS-SOD1 is the abnormal accumulation of conformationally aberrant SOD1 protein (i.e., misfolded SOD1) within motor neurons. Emerging experimental evidence has suggested that misfolded proteins associated with neurodegenerative diseases exhibit prion-like properties, i.e., misfolded proteins act as conformational templates that convert normal proteins into a pathogenic form. Possibly as a result of this prion-like self-propagation property, misfolded forms of pathological proteins are considered to accumulate in the central nervous system and cause neurodegeneration. In this article, we review recent evidence for the role of prion-like mechanisms in ALS-SOD1. In particular, we discuss the propensity of misfolded SOD1 to act as a pathological seed, spread between cells, and propagate neuroanatomically.

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ALS(筋萎縮側索硬化症)もプリオン病と同じ神経変性疾患です。難病に指定されています。 ALSにもイベルメクチンは効果があると特許申請までされ研究が進んでいます。 〈論文〉運動ニューロン疾患 ALS におけるミスフォールドタンパク質のプリオン様特性。現状と今後の展望 https://t.co/SEXOa2LDBj
運動ニューロン疾患 #ALS におけるミスフォールドタンパク質の #プリオン 様特性 現状と今後の展望 徳 田 栄 一,Stefan L. Marklund,古 川 良 明 https://t.co/qwQrGIwnbk

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