Research Articles

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  Research Article

  Correction of Glycogen Synthase Kinase 3β in Myotonic Dystrophy 1 Reduces the Mutant RNA and Improves Postnatal Survival of DMSXL Mice

  Myotonic dystrophy type 1 (DM1) is a multisystem neuromuscular disease without cure. One of the possible therapeutic approaches for DM1 is correction of the RNA-binding proteins CUGBP1 and MBNL1, misregulated in DM1. CUGBP1 activity is controlled by glycogen synthase kinase 3β (GSK3β), which is elevated in skeletal muscle of patients with DM1, and inhibitors of GSK3 were suggested as therapeutic molecules to correct CUGBP1 activity in...

  Mei Wang, Wen-Chin Weng, Lauren Stock, Diana Lindquist, Ana Martinez, Genevieve Gourdon, Nikolai Timchenko, Mike Snape, Lubov Timchenko
• Open Access

  

  Research Article

  1-Palmitoyl-2-Linoleoyl-3-Acetyl-rac-Glycerol Attenuates Streptozotocin-Induced Pancreatic Beta Cell Damage by Promoting Glucose Transporter 2 Endocytosis

  Streptozotocin (STZ) is widely used to induce diabetic rodent models. It is specifically toxic to pancreatic beta cells and causes severe destruction and dysfunction. We investigated the effect of 1-palmitoyl-2-linoleoyl-3-acetyl-rac-glycerol (PLAG) on an STZ-induced diabetic mouse model. PLAG attenuated the glucose increase and maintained serum insulin at levels similar to those seen with control mice.

  Jimin Kim, Joo Heon Kim, Ki-Young Sohn, Sun Young Yoon, Jae Wha Kim
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  Research Article

  PIKfyve Deficiency in Myeloid Cells Impairs Lysosomal Homeostasis in Macrophages and Promotes Systemic Inflammation in Mice

  Macrophages are professional phagocytes that are essential for host defense and tissue homeostasis. Proper membrane trafficking and degradative functions of the endolysosomal system are known to be critical for the function of these cells. We have found that PIKfyve, the kinase that synthesizes the endosomal phosphoinositide phosphatidylinositol-3,5-bisphosphate, is an essential regulator of lysosomal biogenesis and degradative...

  Sang Hee Min, Aae Suzuki, Lehn Weaver, Jessica Guzman, Yutein Chung, Huiyan Jin, Francina Gonzalez, Claire Trasorras, Liang Zhao, Lynn A. Spruce, Steven H. Seeholzer, Edward M. Behrens, Charles S. Abrams
• Open Access

  

  Research Article | Spotlight

  TIN2 Functions with TPP1/POT1 To Stimulate Telomerase Processivity

  TIN2 is an important regulator of telomere length, and mutations in TINF2, the gene encoding TIN2, cause short-telomere syndromes. While the genetics underscore the importance of TIN2, the mechanism through which TIN2 regulates telomere length remains unclear. Here, we tested the effects of human TIN2 on telomerase activity. We identified a new isoform in human cells, TIN2M, that is expressed at levels similar to those of...

  Alexandra M. Pike, Margaret A. Strong, John Paul T. Ouyang, Carol W. Greider