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Publication : Critical role of JSAP1 and JLP in axonal transport in the cerebellar Purkinje cells of mice.

First Author  Sato T Year  2015
Journal  FEBS Lett Volume  589
Issue  19 Pt B Pages  2805-11
PubMed ID  26320416 Mgi Jnum  J:226792
Mgi Id  MGI:5698576 Doi  10.1016/j.febslet.2015.08.024
Citation  Sato T, et al. (2015) Critical role of JSAP1 and JLP in axonal transport in the cerebellar Purkinje cells of mice. FEBS Lett 589(19 Pt B):2805-11
abstractText  JNK/stress-activated protein kinase-associated protein 1 (JSAP1) and JNK-associated leucine zipper protein (JLP) are structurally related scaffolding proteins that are highly expressed in the brain. Here, we found that JSAP1 and JLP play functionally redundant and essential roles in mouse cerebellar Purkinje cell (PC) survival. Mice containing PCs with deletions in both JSAP1 and JLP exhibited PC axonal dystrophy, followed by gradual, progressive neuronal loss. Kinesin-1 cargoes accumulated selectively in the swollen axons of Jsap1/Jlp-deficient PCs. In addition, autophagy inactivation in these mice markedly accelerated PC degeneration. These findings suggest that JSAP1 and JLP play critical roles in kinesin-1-dependent axonal transport, which prevents brain neuronal degeneration.
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