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前頭側頭葉変性症(FTLD)とは,前頭・側頭葉に限局性の萎縮をきたし,行動障害や言語症状などを呈する疾患群の総称である。病理学的には,主としてタウ,TDP-43,FUSのいずれかの細胞内蓄積が認められる。FTLDの症候は多様であるが,近年の病理生化学的および遺伝学的研究の進歩により,臨床-病理-遺伝子の相関関係が明らかになってきており,本症の早期診断法や治療法開発につながると期待される。
Abstract
Frontotemporal lobar degeneration (FTLD) presents diverse clinical symptoms, including psychiatric, behavioral, and language symptoms. Pathologically, it is a collective term of heterogeneous neurodegenerative disorders characterized by deposits of aberrant proteins, including tau, TAR DNA-binding protein of 43kDa (TDP-43), and fused in sarcoma (FUS), predominately in frontotemporal lobes. Recent genetic research has identified several causal and susceptibility genes of FTLD. Moreover, there is an emerging correlation between the clinical-pathological phenotypes and genetic factors. Such knowledge would contribute to further clarification of the pathogenesis of FTLD and the development of novel therapeutic interventions.
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