TY - JOUR
T1 - Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells
T2 - Further evidence that CLN3P has anti-apoptotic properties
AU - Narayan, Srinivas B.
AU - Rakheja, Dinesh
AU - Pastor, Johanne V.
AU - Rosenblatt, Kevin
AU - Greene, Scott R.
AU - Yang, Jichun
AU - Wolf, Bryan A.
AU - Bennett, Michael J.
PY - 2006/6
Y1 - 2006/6
N2 - 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.
AB - 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.
KW - Batten disease
KW - Cytokines
KW - Inherited neurodegenerative disease
KW - Neuronal apoptosis
KW - Neuronal ceroid lipofuscinosis
KW - Pancreatic-derived factor
UR - http://www.scopus.com/inward/record.url?scp=33646419529&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33646419529&partnerID=8YFLogxK
U2 - 10.1016/j.ymgme.2006.01.011
DO - 10.1016/j.ymgme.2006.01.011
M3 - Article
C2 - 16515873
AN - SCOPUS:33646419529
SN - 1096-7192
VL - 88
SP - 178
EP - 183
JO - Molecular genetics and metabolism
JF - Molecular genetics and metabolism
IS - 2
ER -