Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice

• Main Authors: Tea Blom (Author), Mia-Lisa Schmiedt (Author), Andrew M. Wong (Author), Aija Kyttälä (Author), Jarkko Soronen (Author), Matti Jauhiainen (Author), Jaana Tyynelä (Author), Jonathan D. Cooper (Author), Anu Jalanko (Author)
• Format: Book
• Published: The Company of Biologists, 2013-03-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

SUMMARY 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 d...