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Chang, Jae‐,Woong,Choi, Hyunwoo,Cotman, Susan L.,Jung, Yong‐,Keun Blackwell Publishing Ltd 2011 Journal of Neurochemistry Vol.116 No.4
<P> <I>J. Neurochem.</I> (2011) <B>116</B>, 659–668.</P><P><B>Abstract</B></P><P>Juvenile neuronal ceroid lipofuscinosis (Batten disease) is a neurodegenerative disorder caused by mutation in <I>CLN3</I>. Defective autophagy and concomitant accumulation of autofluorescence enriched with mitochondrial ATP synthase subunit c were previously discovered in <I>Cln3</I> mutant knock‐in mice. In this study, we show that treatment with lithium reduces numbers of LC3‐positive autophagosomes and accumulation of LC3‐II in <I>Cln3</I> mutant knock‐in cerebellar cells (Cb<I>Cln3</I><SUP>Δex7/8/Δex7/8</SUP>). Lithium, an inhibitor of GSK3 and IMPase, reduces the accumulation of mitochondrial ATP synthase subunit c and autofluorescence in Cb<I>Cln3</I><SUP>Δex7/8/Δex7/8</SUP> cells, and mitigates the abnormal subcellular distribution of acidic vesicles in the cells. L690,330, an IMPase inhibitor, is as effective as lithium in restoring autophagy in Cb<I>Cln3</I><SUP>Δex7/8/Δex7/8</SUP> cells. Moreover, lithium or down‐regulation of IMPase expression protects Cb<I>Cln3</I><SUP>Δex7/8/Δex7/8</SUP> cells from cell death induced by amino acid deprivation. These results suggest that lithium overcomes the autophagic defect in Cb<I>Cln3</I><SUP>Δex7/8/Δex7/8</SUP> cerebellar cells probably through IMPase, thereby reducing their vulnerability to cell death.</P>
Axonal mRNA in uninjured and regenerating cortical mammalian axons.
Taylor, Anne M,Berchtold, Nicole C,Perreau, Victoria M,Tu, Christina H,Li Jeon, Noo,Cotman, Carl W The Society 2009 The Journal of neuroscience Vol.29 No.15
<P>Using a novel microfluidic chamber that allows the isolation of axons without contamination by nonaxonal material, we have for the first time purified mRNA from naive, matured CNS axons, and identified the presence of >300 mRNA transcripts. We demonstrate that the transcripts are axonal in nature, and that many of the transcripts present in uninjured CNS axons overlap with those previously identified in PNS injury-conditioned DRG axons. The axonal transcripts detected in matured cortical axons are enriched for protein translational machinery, transport, cytoskeletal components, and mitochondrial maintenance. We next investigated how the axonal mRNA pool changes after axotomy, revealing that numerous gene transcripts related to intracellular transport, mitochondria and the cytoskeleton show decreased localization 2 d after injury. In contrast, gene transcripts related to axonal targeting and synaptic function show increased localization in regenerating cortical axons, suggesting that there is an increased capacity for axonal outgrowth and targeting, and increased support for synapse formation and presynaptic function in regenerating CNS axons after injury. Our data demonstrate that CNS axons contain many mRNA species of diverse functions, and suggest that, like invertebrate and PNS axons, CNS axons synthesize proteins locally, maintaining a degree of autonomy from the cell body.</P>