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Neuronal ceroid lipofuscinosis related ER membrane protein CLN8 regulates PP2A activity and ceramide levels.

Biochim Biophys Acta Mol Basis Dis. 2019; 
AdhikariBabita,De SilvaBhagya,MolinaJoshua A,AllenAshton,PeckSun H,LeeStel
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Monoclonal Antibody Services ACCEPTED MANUSCRIPT ACCEPTED MANUSCRIPT Materials and Methods Antibodies Mouse monoclonal antibodies used in this study were against Myc epitope (9E10, hybridoma cell line from ATCC (CRL 1729)), GAPDH (Developmental Studies Hybridoma Bank (DSHB), 4B7), tubulin (DSHB, 12G10), beta-actin (GenScript, A00702). Get A Quote

摘要

The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative lysosomal storage disorders. CLN8 deficiency causes a subtype of NCL, referred to as CLN8 disease. CLN8 is an ER resident protein with unknown function; however, a role in ceramide metabolism has been suggested. In this report, we identified PP2A and its biological inhibitor I2PP2A as interacting proteins of CLN8. PP2A is one of the major serine/threonine phosphatases in cells and governs a wide range of signaling pathways by dephosphorylating critical signaling molecules. We showed that the phosphorylation levels of several substrates of PP2A, namely Akt, S6 kinase, and GSK3β, were decreased in CLN8 disease pati... More

关键词

CLN8,Ceramide,I2PP2A,Neuronal ceroid lipofuscinosis,