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Brain Advance Access published online on July 7, 2004
Brain, doi:10.1093/brain/awh195
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Received October 21, 2003
Revised March 20, 2004
Accepted March 22, 2004

Article

CLN3L, a novel protein related to the Batten disease protein, is overexpressed in Cln3-/- mice and in Batten disease

Srinivas B. Narayan 1, Johanne V. Pastor 1, Hannah M. Mitchison 2, Michael J. Bennett 1*

1 Department of Pathology and Pediatrics, University of Texas Southwestern Medical Center at Dallas, Dallas, USA
2 Department of Paediatrics and Child Health, Royal Free and University College Medical School, London, UK

* To whom correspondence should be addressed. E-mail: michaelj.bennett{at}utsouthwestern.edu.


  Abstract

Summary Batten disease is a severe autosomal recessive neurodegenerative disease which results from mutations in CLN3. Although the gene was cloned in 1995, the tissue distribution and subcellular localization of the CLN3 protein (CLN3P) remains inconclusive. We have demonstrated the presence of a novel 33 kDa protein in both normal human and wild-type mouse brain. This 33 kDa protein, which is overexpressed in brains of patients with Batten disease and in Cln3-/- mouse brain, binds to the antibody raised against the peptide sequence of CLN3P and results in aberrant CLN3P localization studies. We expressed a novel 33 kDa protein that is highly similar to CLN3P. We showed that the 33 kDa protein is identical to that recognized in Batten disease and Cln3-/- brain. These studies strongly suggest the presence of an alternative CLN3-like (CLN3L) product in Batten disease. Previous studies of CLN3P tissue distribution and intracellular localization will require extensive reanalysis in order to determine the true expression of CLN3P.

Keywords: Batten disease; CLN3 protein; Cln3 gene; cln3-/- mice; neurodegenerative disease.
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