Article
Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.
Disease models & mechanisms - 1 Mar 2013
Blom Tea, Schmiedt Mia-Lisa, Wong Andrew M, Kyttälä Aija, Soronen Jarkko, Jauhiainen Matti, Tyynelä Jaana, Cooper Jonathan D, Jalanko Anu
Abstract excerpt
Both CLN1 and CLN5 deficiencies lead to severe neurodegenerative diseases of childhood, known as neuronal ceroid lipofuscinoses (NCLs). The broadly similar phenotypes of NCL mouse models, and the potential for interactions between NCL proteins, raise the possibility of shared or convergent disease mechanisms. To begin addressing these issues, we have developed a new mouse model lacking both Cln1 and Cln5 genes....
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