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Autor:
Eamonn Sheridan, Ellanor L. Whiteley, Tarek Z. Deeb, Roland Bürli, Nicholas J. Brandon, Gonzalo S. Tejeda, George S. Baillie, Stephen J. Moss
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America
Significance Phosphodiesterase 10A (PDE10A) is as a target of interest in Huntington’s disease (HD) as levels of the enzyme have been shown to decrease prior to the development of the hallmark motor symptoms. Clearly, a better understanding of how
Protrudin binds atlastins and endoplasmic reticulum-shaping proteins and regulates network formation
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 110(37)
Hereditary spastic paraplegias are inherited neurological disorders characterized by progressive lower-limb spasticity and weakness. Although more than 50 genetic loci are known [spastic gait (SPG)1 to -57], over half of hereditary spastic paraplegia
HSV carrying WT REST establishes latency but reactivates only if the synthesis of REST is suppressed
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 110(6)
HSVs transit from vigorous replication at the portal of entry into the body to a latent state in sensory neurons in which only noncoding (e.g., latency-associated transcript) and micro-RNAs are expressed. In productive infection, viral genes must be
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 108(31)
GPR56, an orphan G protein-coupled receptor (GPCR) from the family of adhesion GPCRs, plays an indispensable role in cortical development and lamination. Mutations in the GPR56 gene cause a malformed cerebral cortex in both humans and mice that resem
Autor:
Shivakumar Vasanth, Jonathan M. Shillingford, Nicholas Doerr, Thomas Weimbs, Terry Watnick, Mike T. Kinter, Jeffrey J. Talbot, Sambuddho Mukherjee
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 108(19)
Mutations in polycystin-1 (PC1) lead to autosomal-dominant polycystic kidney disease (ADPKD), a leading cause of renal failure for which no treatment is available. PC1 is an integral membrane protein, which has been implicated in the regulation of mu
Autor:
Jeffrey S. Chamberlain, Jay Volinski, Jaclyn Schienda, Antonio L. Perez, Louis M. Kunkel, Estanislao Bachrach, Sheng Li, Kalliopi Liadaki, Alan J. Flint
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 101(10)
Cell-based therapy for Duchenne muscular dystrophy patients and mdx mice has proven to be a safe but ineffective form of treatment. Recently, a group of cells called muscle side population (SP) cells have been isolated based on their ability to efflu
Autor:
Robert F. Kalejta, Thomas Shenk
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 100(6)
Most of the substrates degraded by the proteasome are marked with polyubiquitin chains. However, there are a limited number of examples of nonubiquitinated proteins that are degraded by the proteasome. Here, we describe the degradation of the retinob
Autor:
Yun Wang, Andreas Tomac, YaJun Zhang, Cristina Bäckman, Heiner Westphal, Marisela Morales, Norman J. Haughey, C.F. Chang, Alan D. Agulnick, Mark P. Mattson, Barry J. Hoffer
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 99(14)
Persephin (Pspn), a recently cloned member of the transforming growth factor-β superfamily (TGF-β) and glial cell line-derived neurotrophic factor (GDNF) subfamily, is distributed throughout the nervous system at extremely low levels and is thought
Autor:
Hans Lehrach, Gillian P. Bates, Katja Schweiger, Renate Hasenbank, Eberhard Scherzinger, Annie Sittler, Rudi Lurz, Erich E. Wanker, Volker Heiser
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 96(8)
Huntington’s disease is a progressive neurodegenerative disorder caused by a polyglutamine [poly(Q)] repeat expansion in the first exon of the huntingtin protein. Previously, we showed that N-terminal huntingtin peptides with poly(Q) tracts in the
Autor:
Cornelis Murre, Isaac Engel
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 96(3)
Mice with null mutations in the E2A gene are highly susceptible to the spontaneous development of thymic lymphomas. To understand better how E2A deficiency may contribute to lymphomagenesis, we have observed the consequences of enforced expression of