Survival motor neuron loss in motor neuron disease
Grant number: 1008910 | Funding period: 2011 - 2014
The aim of this proposal is to understand the earliest and central pathological events in motor neuron disease (MND). This is important for drug development because therapies that target the early disease stages are likely to be effective. Our previous studies suggest that a protein essential for nerve cells, survival motor neuron, is lacking in MND. We will therefore examine how early this deficiency starts in disease and whether its replacement is beneficial. These outcomes may aid future studies to design new treatments for MND.
Related publications (10)
SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation
Rafaa Zeineddine, Jay F Pundavela, Lisa Corcoran, Elise M Stewart, Dzung Do-Ha, Monique Bax, Gilles Guillemin, Kara L Vine, Danny M Hatters, Heath Ecroyd, Christopher M Dobson, Bradley J Turner, Lezanne Ooi, Mark R Wilson, Neil R Cashman, Justin J Yerbury
BACKGROUND: Amyotrophic Lateral Sclerosis is characterized by a focal onset of symptoms followed by a progressive spread of pathol..
Effect of thymic stimulation of CD4 T cell expansion on disease onset and progression in mutant SOD1 mice
Rebecca K Sheean, Richard H Weston, Nirma D Perera, Angela D'Amico, Stephen L Nutt, Bradley J Turner
BACKGROUND: The peripheral immune system is implicated in modulating microglial activation, neurodegeneration and disease progress..
The Hippo pathway effector YAP is a critical regulator of skeletal muscle fibre size
KI Watt, BJ Turner, A Hagg, X Zhang, JR Davey, H Qian, C Beyer, CE Winbanks, KF Harvey, P Gregorevic
The Yes-associated protein (YAP) is a core effector of the Hippo pathway, which regulates proliferation and apoptosis in organ dev..
Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice
Bradley J Turner, Neza Alfazema, Rebecca K Sheean, James N Sleigh, Kay E Davies, Malcolm K Horne, Kevin Talbot
Spinal muscular atrophy results from diminished levels of survival motor neuron (SMN) protein in spinal motor neurons. Low levels ..
Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms
Leslie I Grad, Justin J Yerbury, Bradley J Turner, William C Guest, Edward Pokrishevsky, Megan A O'Neill, Anat Yanai, Judith M Silverman, Rafaa Zeineddine, Lisa Corcoran, Janet R Kumita, Leila M Luheshi, Masoud Yousefi, Bradley M Coleman, Andrew F Hill, Steven S Plotkin, Ian R Mackenzie, Neil R Cashman
Amyotrophic lateral sclerosis (ALS) is predominantly sporadic, but associated with heritable genetic mutations in 5-10% of cases, ..
The bone morphogenetic protein axis is a positive regulator of skeletal muscle mass
Catherine E Winbanks, Justin L Chen, Hongwei Qian, Yingying Liu, Bianca C Bernardo, Claudia Beyer, Kevin I Watt, Rachel E Thomson, Timothy Connor, Bradley J Turner, Julie R McMullen, Lars Larsson, Sean L McGee, Craig A Harrison, Paul Gregorevic
Although the canonical transforming growth factor β signaling pathway represses skeletal muscle growth and promotes muscle wasting..
Dysregulation of the complement cascade in the hSOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis
John D Lee, Nur A Kamaruzaman, Jenny NT Fung, Stephen M Taylor, Bradley J Turner, Julie D Atkin, Trent M Woodruff, Peter G Noakes
BACKGROUND: Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral scle..
CO-REGULATION OF SURVIVAL OF MOTOR NEURON AND BCL-XL EXPRESSION: IMPLICATIONS FOR NEUROPROTECTION IN SPINAL MUSCULAR ATROPHY
RS Anderton, LL Price, BJ Turner, BP Meloni, C Mitrpant, FL Mastaglia, C Goh, SD Wilton, S Boulos
Spinal muscular atrophy (SMA), a fatal genetic motor disorder of infants, is caused by diminished full-length survival of motor ne..