NCL RESOURCE - A GATEWAY FOR BATTEN DISEASE

Mouse Models

Mouse models have been developed for many diseases. Two mouse models for NCL are naturally occurring, the remainder have been engineered. Some mouse models have mutations in genes not known to cause disease in human patients. Studying mouse models for NCL allows further understanding of the disease process.

For detailed information, see Mouse Models Table, maintained by Hannah Mitchison.

Mouse Genes Tables

• Cln1 - 2 engineered models for CLN1/PPT1 exist.
• Cln2 - 2 engineered models for CLN2/TPP1 exists.
• Cln3 - 4 engineered models for CLN3 exist.
• Cln5 - 1 engineered model for CLN5 exists.
• cln6/nclf - 1 naturally occurring model for CLN6 exists.
• cln8/mnd - 1 naturally occurring model for CLN8 exists.
• Ctsd - 1 engineered model for CTSD exists.
• Clcn3 - 1 engineered model with a mutation in ClC-3 resembles NCL.
• Clcn6 - 1 engineered model with a mutation in ClC-6 resembles NCL.
• Clcn7 - 1 engineered model with a mutation in ClC-7 has osteopetrosis and features that resembles NCL.
• grey lethal - 1 engineered model with a mutation in Ostm1, which associates with ClC-7, has osteopetrosis and features that resembles NCL.
• Ctsf - 1 engineered model with a mutation in CTSF resembles NCL.
• Ctsb/l - 1 engineered model lacking both CTSB and CTSL resembles NCL.

'NCL Resource' is maintained by Sara Mole.

MRC Laboratory for Molecular Cell Biology, University College London, Gower Street, London. WC1E 6BT. United Kingdom.
If you have comments or suggestions or details for inclusion, email ncl-www@ucl.ac.uk or telephone +44 207 679 7257.
Last updated 16/10/2018