Literature DB >> 33659294

Generation, Analyzing and in-vivo Drug Treatment of Drosophila Models with IBMPFD.

Ting Zhang1, Bruce A Hay2, Ming Guo1,3,4.   

Abstract

Missense mutations of p97/cdc48/Valosin-containing protein (VCP) cause inclusion body myopathy, Paget disease with frontotemporal dementia (IBMPFD) and other neurodegenerative diseases. The pathological mechanism of IBMPFD is not clear and there is no treatment. We generated Drosophila models of IBMPFD in adult flight muscle in vivo. Here we describe a variety of assays to characterize disease pathology and dissect disease mechanism, and the consequences of in vivo feeding of VCP inhibitors.
Copyright © The Authors; exclusive licensee Bio-protocol LLC.

Entities:  

Keywords:  Disease models; Drosophila; Drug treatment; Inclusion body myopathy; Inhibitors; Mitochondria; Muscle; Paget disease and frontotemporal dementia (IBMPFD); VCP/p97

Year:  2020        PMID： 33659294      PMCID： PMC7842768          DOI： 10.21769/BioProtoc.3621

Source DB:  PubMed          Journal:  Bio Protoc        ISSN： 2331-8325


  22 in total

1.  The Parkinson's disease genes pink1 and parkin promote mitochondrial fission and/or inhibit fusion in Drosophila.

Authors:  Hansong Deng; Mark W Dodson; Haixia Huang; Ming Guo
Journal:  Proc Natl Acad Sci U S A       Date:  2008-09-17       Impact factor: 11.205

Review 2.  Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system.

Authors:  Hemmo Meyer; Monika Bug; Sebastian Bremer
Journal:  Nat Cell Biol       Date:  2012-02-02       Impact factor: 28.824

3.  Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations.

Authors:  Danilo Ritz; Maja Vuk; Philipp Kirchner; Monika Bug; Sabina Schütz; Arnold Hayer; Sebastian Bremer; Caleb Lusk; Robert H Baloh; Houkeun Lee; Timo Glatter; Matthias Gstaiger; Ruedi Aebersold; Conrad C Weihl; Hemmo Meyer
Journal:  Nat Cell Biol       Date:  2011-08-07       Impact factor: 28.824

4.  Drosophila pink1 is required for mitochondrial function and interacts genetically with parkin.

Authors:  Ira E Clark; Mark W Dodson; Changan Jiang; Joseph H Cao; Jun R Huh; Jae Hong Seol; Soon Ji Yoo; Bruce A Hay; Ming Guo
Journal:  Nature       Date:  2006-05-03       Impact factor: 49.962

5.  TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

Authors:  Gillian P Ritson; Sara K Custer; Brian D Freibaum; Jake B Guinto; Dyanna Geffel; Jennifer Moore; Waixing Tang; Matthew J Winton; Manuela Neumann; John Q Trojanowski; Virginia M-Y Lee; Mark S Forman; J Paul Taylor
Journal:  J Neurosci       Date:  2010-06-02       Impact factor: 6.167

6.  VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations.

Authors:  Nam Chul Kim; Emilie Tresse; Regina-Maria Kolaitis; Amandine Molliex; Ruth E Thomas; Nael H Alami; Bo Wang; Aashish Joshi; Rebecca B Smith; Gillian P Ritson; Brett J Winborn; Jennifer Moore; Joo-Yong Lee; Tso-Pang Yao; Leo Pallanck; Mondira Kundu; J Paul Taylor
Journal:  Neuron       Date:  2013-03-14       Impact factor: 17.173

Review 7.  VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder.

Authors:  Virginia E Kimonis; Erin Fulchiero; Jouni Vesa; Giles Watts
Journal:  Biochim Biophys Acta       Date:  2008-09-18

8.  Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein.

Authors:  Giles D J Watts; Jill Wymer; Margaret J Kovach; Sarju G Mehta; Steven Mumm; Daniel Darvish; Alan Pestronk; Michael P Whyte; Virginia E Kimonis
Journal:  Nat Genet       Date:  2004-03-21       Impact factor: 38.330

9.  Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis.

Authors:  Yevgeniya Abramzon; Janel O Johnson; Sonja W Scholz; J P Taylor; Maura Brunetti; Andrea Calvo; Jessica Mandrioli; Michael Benatar; Gabriele Mora; Gabriella Restagno; Adriano Chiò; Bryan J Traynor
Journal:  Neurobiol Aging       Date:  2012-05-08       Impact factor: 4.673

10.  MUL1 acts in parallel to the PINK1/parkin pathway in regulating mitofusin and compensates for loss of PINK1/parkin.

Authors:  Jina Yun; Rajat Puri; Huan Yang; Michael A Lizzio; Chunlai Wu; Zu-Hang Sheng; Ming Guo
Journal:  Elife       Date:  2014-06-04       Impact factor: 8.140
View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.