A novel neuroferritinopathy mouse model (FTL 498InsTC) shows progressive brain iron dysregulation, morphological signs of early neurodegeneration and motor coordination deficits
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- 作者：Federica Maccarinellia ; 1 ; Antonella Paganib ; 1 ; Anna Cozzib ; c ; Franca Codazzib ; Giuseppina Di Giacomoc ; Sara Capocciad ; Stefania Rapinoe ; Dario Finazzia ; Letterio Salvatore Politib ; Francesca Cirullid ; Marco Giorgioe ; Ottavio Cremonab ; c ; Fabio Grohovazb ; c ; 2 ; grohovaz.fabio@hsr.it" class="auth_mail" title="E-mail the corresponding author ; Sonia Levib ; c ; 2 ; levi.sonia@hsr.it" class="auth_mail" title="E-mail the corresponding author
- 关键词：NF ; neuroferritinopathy ; FTL ; ferritin light chain gene ; FtL ; ferritin light chain protein ; FtH ; ferritin heavy chain protein ; Tg ; transgenic ; WT ; wild type ; ROS ; reactive oxygen species
- 刊名：Neurobiology of Disease
- 出版年：2015
- 出版时间：September 2015
- 年：2015
- 卷：81
- 期：Complete
- 页码：119-133
- 全文大小：3193 K
文摘
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We developed two new neuroferritinopathy mice models (NF).
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NF brains are characterized by iron/ferritin accumulation and oxidative damage.
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NF brains show granules of lipofuscine associated with iron.
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A mechanism of lipofuscine formation is proposed.
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NF mice show impaired motor coordination increasing with age.