| First Author | Lim MJ | Year | 2007 |
| Journal | Neurobiol Dis | Volume | 25 |
| Issue | 2 | Pages | 239-51 |
| PubMed ID | 17070688 | Mgi Jnum | J:134735 |
| Mgi Id | MGI:3789746 | Doi | 10.1016/j.nbd.2006.09.005 |
| Citation | Lim MJ, et al. (2007) IgG entry and deposition are components of the neuroimmune response in Batten disease. Neurobiol Dis 25(2):239-51 |
| abstractText | Patients and a mouse model of Batten disease, the juvenile form of neuronal ceroid lipofuscinosis (JNCL), raise autoantibodies against GAD65 and other brain-directed antigens. Here we investigate the adaptive component of the neuroimmune response. Cln3(-/-) mice have autoantibodies to GAD65 in their cerebrospinal fluid and elevated levels of brain bound immunoglobulin G (IgG). IgG deposition was found within human JNCL autopsy material, a feature that became more evident with increased age in Cln3(-/-) mice. The lymphocyte infiltration present in human and murine JNCL occurred late in disease progression, and was not capable of central/intrathecal IgG production. In contrast, we found evidence for an early systemic immune dysregulation in Cln3(-/-) mice. In addition evidence for a size-selective breach in the blood-brain barrier integrity in these mice suggests that systemically produced autoantibodies can access the JNCL central nervous system and contribute to a progressive inflammatory response. |
