Zobrazeno 1 - 10
of 279
pro vyhledávání: '"Gillian P Bates"'
Autor:
Karlijne W Geijtenbeek, Jolien Janzen, Aleksandra E Bury, Alicia Sanz-Sanz, Ron A Hoebe, Marie K Bondulich, Gillian P Bates, Eric A J Reits, Sabine Schipper-Krom
Publikováno v:
PLoS ONE, Vol 17, Iss 12, p e0278130 (2022)
Huntington's disease is an autosomal dominant heritable disorder caused by an expanded CAG trinucleotide repeat at the N-terminus of the Huntingtin (HTT) gene. Lowering the levels of soluble mutant HTT protein prior to aggregation through increased d
Externí odkaz:
https://doaj.org/article/ab2a3b5b3aba44b890ddb44b5ee0aee0
Autor:
Wolfgang Reindl, Barbara Baldo, Jana Schulz, Isabell Janack, Ilka Lindner, Markus Kleinschmidt, Yalda Sedaghat, Christina Thiede, Karsten Tillack, Christina Schmidt, Isabell Cardaun, Tom Schwagarus, Frank Herrmann, Madlen Hotze, Georgina F Osborne, Simone Herrmann, Andreas Weiss, Celina Zerbinatti, Gillian P Bates, Jonathan Bard, Ignacio Munoz-Sanjuan, Douglas Macdonald
Publikováno v:
PLoS ONE, Vol 14, Iss 3, p e0213521 (2019)
Huntington's disease (HD) is a monogenic neurodegenerative disorder caused by an expansion of the CAG trinucleotide repeat domain in the huntingtin (HTT) gene, leading to an expanded poly-glutamine (polyQ) stretch in the HTT protein. This mutant HTT
Externí odkaz:
https://doaj.org/article/1d1d996e734c4cbdb4d4b5b4a98abcb8
Autor:
Ivan Rattray, Edward J Smith, William R Crum, Thomas A Walker, Richard Gale, Gillian P Bates, Michel Modo
Publikováno v:
PLoS ONE, Vol 12, Iss 1, p e0168556 (2017)
A variety of mouse models have been developed that express mutant huntingtin (mHTT) leading to aggregates and inclusions that model the molecular pathology observed in Huntington's disease. Here we show that although homozygous HdhQ150 knock-in mice
Externí odkaz:
https://doaj.org/article/02a1eb9f87a24ff0bf3fe0e6e3785ecf
Autor:
Raffaella Tulino, Agnesska C Benjamin, Nelly Jolinon, Donna L Smith, Eduardo N Chini, Alisia Carnemolla, Gillian P Bates
Publikováno v:
PLoS ONE, Vol 11, Iss 2, p e0150682 (2016)
[This corrects the article DOI: 10.1371/journal.pone.0145425.].
Externí odkaz:
https://doaj.org/article/243117c9159c4daaba9f9a69f9732654
Autor:
Raffaella Tulino, Agnesska C Benjamin, Nelly Jolinon, Donna L Smith, Eduardo N Chini, Alisia Carnemolla, Gillian P Bates
Publikováno v:
PLoS ONE, Vol 11, Iss 1, p e0145425 (2016)
Huntington's disease (HD) is a neurodegenerative disorder for which there are no disease-modifying treatments. SIRT1 is a NAD+-dependent protein deacetylase that is implicated in maintaining neuronal health during development, differentiation and age
Externí odkaz:
https://doaj.org/article/fa4ad6c77f364fdf81ee63cfbb192c31
Autor:
Michal Mielcarek, Marta Toczek, Cleo J L M Smeets, Sophie A Franklin, Marie K Bondulich, Nelly Jolinon, Thomas Muller, Mhoriam Ahmed, James R T Dick, Izabela Piotrowska, Linda Greensmith, Ryszard T Smolenski, Gillian P Bates
Publikováno v:
PLoS Genetics, Vol 11, Iss 3, p e1005021 (2015)
Skeletal muscle remodelling and contractile dysfunction occur through both acute and chronic disease processes. These include the accumulation of insoluble aggregates of misfolded amyloid proteins that is a pathological feature of Huntington's diseas
Externí odkaz:
https://doaj.org/article/ce3b43e9c4de4c43aae30f8c307eef76
Publikováno v:
PLoS ONE, Vol 10, Iss 7, p e0131985 (2015)
The heat shock response (HSR) is the main pathway used by cells to counteract proteotoxicity. The inability of differentiated neurons to induce an HSR has been documented in primary neuronal cultures and has been proposed to play a critical role in a
Externí odkaz:
https://doaj.org/article/29208f9844014aa5b5a4ef92480aace3
Autor:
Ruth Luthi-Carter, Barbara L Apostol, Anthone W Dunah, Molly M DeJohn, Laurie A Farrell, Gillian P Bates, Anne B Young, David G Standaert, Leslie M Thompson, Jang-Ho J Cha
Publikováno v:
Neurobiology of Disease, Vol 14, Iss 3, Pp 624-636 (2003)
We analyzed NMDA receptor subunit mRNAs, proteins, and anchoring proteins in mice transgenic for exon 1 of the HD gene. R6/2 mice had decreased levels of mRNAs encoding ε1 and ε2 NMDA receptor subunits (mouse orthologs of rat NR2A and NR2B subunits
Externí odkaz:
https://doaj.org/article/4e6b60ce52e44b97b00d9332a0e9f1f1
Autor:
Michal Mielcarek, Linda Inuabasi, Marie K Bondulich, Thomas Muller, Georgina F Osborne, Sophie A Franklin, Donna L Smith, Andreas Neueder, Jim Rosinski, Ivan Rattray, Andrea Protti, Gillian P Bates
Publikováno v:
PLoS Genetics, Vol 10, Iss 8, p e1004550 (2014)
Cardiac remodelling and contractile dysfunction occur during both acute and chronic disease processes including the accumulation of insoluble aggregates of misfolded amyloid proteins that are typical features of Alzheimer's, Parkinson's and Huntingto
Externí odkaz:
https://doaj.org/article/999732e06288452195737c7385566612
Autor:
Silvia Corrochano, Maurizio Renna, Georgina Osborne, Sarah Carter, Michelle Stewart, Joel May, Gillian P Bates, Steve D M Brown, David C Rubinsztein, Abraham Acevedo-Arozena
Publikováno v:
PLoS ONE, Vol 9, Iss 8, p e105595 (2014)
Many of the neurodegenerative diseases that afflict people in later life are associated with the formation of protein aggregates. These so-called "proteinopathies" include Alzheimer's disease (AD) and Huntington's disease (HD). The insulin/insulin-li
Externí odkaz:
https://doaj.org/article/43c58186b4ad4a72a1ba864ebed82208