SIRT1 activation rescues the mislocalization of RNA-binding proteins and cognitive defects induced by inherited cobalamin disorders.

Rose Ghemrawi,Carole Arnold,Shyue-Fang Battaglia-Hsu,Grégory Pourié, Isabelle Trinh, Christine Bassila,Rashka Charif,Arnaud Wiedemann,Justine Flayac,Aurélie Robert,Natacha Dreumont,François Feillet,Jean-Louis Guéant,David Coelho

Metabolism（2019）

引用 23|浏览27

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摘要

•Defects of cobalamin metabolism lead to mislocalization of RNA binding proteins.•SRT1720 treatment rescued abnormal localization of RNA binding proteins.•SRT1720 treatment improved the cognitive defects observed in a transgenic mouse model.

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ABCD4,AdoCbl,Adox,ATF6,ELAVL1,ER,FISH,HnRNPA1,HSF1,HuR,IRF3,LMBD1,MeCbl,MTR,MTRR,OA,PLA,PP2A,RBM10,RBP,SAM,SIRT1,SRSF1,SSC,TG

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