Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: Further evidence that CLN3P has anti-apoptotic properties

Srinivas B. Narayan, Dinesh Rakheja, Johanne V. Pastor, Kevin Rosenblatt, Scott R. Greene, Jichun Yang, Bryan A. Wolf, Michael J. Bennett

Research output: Contribution to journalArticle

25 Scopus citations

Abstract

Juvenile neuronal ceroid-lipofuscinosis (JNCL) or Batten/Spielmeyer-Vogt-Sjogren disease (OMIM #204200) is one of a group of nine clinically related inherited neurodegenerative disorders (CLN1-9). JNCL results from mutations in CLN3 on chromosome 16p12.1. The neuronal loss in Batten disease has been shown to be due to a combination of apoptosis and autophagy suggesting that CLN3P, the defective protein, may have an anti-neuronal death function. PANDER (PANcreatic-DERived factor) is a novel cytokine that was recently cloned from pancreatic islet cells. PANDER is specifically expressed in the pancreatic islets, small intestine, testis, prostate, and neurons of the central nervous system, and has been demonstrated to induce apoptosis. In this study, we over-expressed CLN3P in SH-SY5Y neuroblastoma cells and monitored the effects on PANDER-induced apoptosis. CLN3P significantly increased the survival rate of the SH-SY5Y cells in this system. This study provides additional evidence that the function of CLN3P is related to preventing neuronal apoptosis.

Original languageEnglish (US)
Pages (from-to)178-183
Number of pages6
JournalMolecular genetics and metabolism
Volume88
Issue number2
DOIs
StatePublished - Jun 1 2006

Keywords

  • Batten disease
  • Cytokines
  • Inherited neurodegenerative disease
  • Neuronal apoptosis
  • Neuronal ceroid lipofuscinosis
  • Pancreatic-derived factor

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Endocrinology

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