title: Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: further evidence that CLN3P has anti-apoptotic properties.
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.
authors.affiliation: Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Srinivas B
authors.initials: SB
authors.lastname: Narayan
authors.forename: Dinesh
authors.initials: D
authors.lastname: Rakheja
authors.forename: Johanne V
authors.initials: JV
authors.lastname: Pastor
authors.forename: Kevin
authors.initials: K
authors.lastname: Rosenblatt
authors.forename: Scott R
authors.initials: SR
authors.lastname: Greene
authors.forename: Jichun
authors.initials: J
authors.lastname: Yang
authors.forename: Bryan A
authors.initials: BA
authors.lastname: Wolf
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Bennett
mesh_terms: D017209:Apoptosis; D051017:Apoptosis Regulatory Proteins / Q000235:genetics / Q000502:physiology*; D045744:Cell Line, Tumor; D016207:Cytokines / Q000235:genetics / Q000502:physiology*; D006801:Humans; D008562:Membrane Glycoproteins / Q000235:genetics / Q000502:physiology*; D018832:Molecular Chaperones / Q000235:genetics / Q000502:physiology*; D009447:Neuroblastoma; D009472:Neuronal Ceroid-Lipofuscinoses / Q000209:etiology*; D009474:Neurons / Q000166:cytology*; D040081:Protein Array Analysis; D014162:Transfection
chemical_list: D051017:Apoptosis Regulatory Proteins; C105199:CLN3 protein, human; D016207:Cytokines; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones