Literature DB >> 14729833

Congo red, doxycycline, and HSP70 overexpression reduce aggregate formation and cell death in cell models of oculopharyngeal muscular dystrophy.

Y P Bao, S Sarkar, E Uyama, D C Rubinsztein.   

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Year:  2004        PMID: 14729833      PMCID: PMC1757258          DOI: 10.1136/jmg.2003.014548

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


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  9 in total

1.  Progressive myopathy in an inducible mouse model of oculopharyngeal muscular dystrophy.

Authors:  Ami Mankodi; Thurman M Wheeler; Reena Shetty; Kelly M Salceies; Mark W Becher; Charles A Thornton
Journal:  Neurobiol Dis       Date:  2011-09-21       Impact factor: 5.996

2.  Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 protein.

Authors:  Vered Raz; Samantha Routledge; Andrea Venema; Hellen Buijze; Erik van der Wal; Seyedyahya Anvar; Kirsten R Straasheijm; Rinse Klooster; Michael Antoniou; Silvère M van der Maarel
Journal:  Am J Pathol       Date:  2011-08-18       Impact factor: 4.307

3.  Doxycycline attenuates protein aggregation in cardiomyocytes and improves survival of a mouse model of cardiac proteinopathy.

Authors:  Hanqiao Zheng; Mingxin Tang; Qingwen Zheng; Asangi R K Kumarapeli; Kathleen M Horak; Zongwen Tian; Xuejun Wang
Journal:  J Am Coll Cardiol       Date:  2010-10-19       Impact factor: 24.094

4.  Assessment of PABPN1 nuclear inclusions on a large cohort of patients and in a human xenograft model of oculopharyngeal muscular dystrophy.

Authors:  Fanny Roth; Jamila Dhiab; Alexis Boulinguiez; Hadidja-Rose Mouigni; Saskia Lassche; Elisa Negroni; Laura Muraine; Alix Marhic; Alison Oliver; Jeanne Lainé; Andrée Rouche; Erin K O'Ferrall; Baziel van Engelen; Coen Ottenheijm; Hagar Greif; Sergiu Blumen; Jean Lacau St Guily; Sophie Perie; Gillian Butler-Browne; Vincent Mouly; Capucine Trollet
Journal:  Acta Neuropathol       Date:  2022-10-05       Impact factor: 15.887

Review 5.  Oculopharyngeal muscular dystrophy: a polyalanine myopathy.

Authors:  Bernard Brais
Journal:  Curr Neurol Neurosci Rep       Date:  2009-01       Impact factor: 5.081

Review 6.  PABPN1: molecular function and muscle disease.

Authors:  Ayan Banerjee; Luciano H Apponi; Grace K Pavlath; Anita H Corbett
Journal:  FEBS J       Date:  2013-05-24       Impact factor: 5.542

7.  A folded and functional protein domain in an amyloid-like fibril.

Authors:  Mirko Sackewitz; Sabrina von Einem; Gerd Hause; Michael Wunderlich; Franz-Xaver Schmid; Elisabeth Schwarz
Journal:  Protein Sci       Date:  2008-04-18       Impact factor: 6.725

8.  Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy.

Authors:  Nicolas Barbezier; Aymeric Chartier; Yannick Bidet; Anja Buttstedt; Cécile Voisset; Hervé Galons; Marc Blondel; Elisabeth Schwarz; Martine Simonelig
Journal:  EMBO Mol Med       Date:  2011-01       Impact factor: 12.137

9.  The Inhibition of Heat Shock Protein 90 Facilitates the Degradation of Poly-Alanine Expanded Poly (A) Binding Protein Nuclear 1 via the Carboxyl Terminus of Heat Shock Protein 70-Interacting Protein.

Authors:  Chao Shi; Xuan Huang; Bin Zhang; Dan Zhu; Huqiao Luo; Quqin Lu; Wen-Cheng Xiong; Lin Mei; Shiwen Luo
Journal:  PLoS One       Date:  2015-09-28       Impact factor: 3.240

  9 in total

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