59 results on '"Greensmith L"'
Search Results
2. 241st ENMC international workshop: Towards a European unifying lab for Kennedy's disease. 15–17th February, 2019 Hoofddorp, The Netherlands
3. Muscle and neuronal peripheral biomarkers for spinal and bulbar muscle atrophy: muscle holds more promise
4. Do diverse Charcot-Marie-Tooth disease-causing mutations show convergent disease pathomechanisms? Investigation of mitochondrial dysfunction in CMT
5. Region specific glial stress responses: implications for ALS
6. Establishing motor neuron and astrocytic cultures to study spinal and bulbar muscular atrophy
7. Dissecting TDP-43 gain- and loss-of-function in neurodegeneration
8. Mitochondrial dysfunction and abnormal calcium handling in cell models of Hereditary Sensory Neuropathy type I
9. Using stem cells and light to restore function to paralysed muscles
10. Investigating inherent cellular changes in patient-derived mutant VCP fibroblasts as a model of inclusion body myopathy
11. Characterisation of the heat shock response in primary and ES-cell derived spinal motor neurons and astrocytes exposed to cellular stress conditions modelling ALS
12. Modulation of cytoplasmic dynein and tubulin modification as potential therapeutic targets in SMA-LED
13. Investigating dysfunctional RNA processing in TDP-43 mouse mutants
14. In vitro models of Charcot-Marie-Tooth disease for investigating disease pathomechanisms
15. Neuropathological findings from a human post mortem case of distal hereditary motor neuropathy (dHMN) due to p.Ser135Phe HSPB1 mutation and transgenic mice with mutant or wild-type HSP27 overexpression
16. P49 Mitochondrial abnormalities and increased oxidative stress in HSBP1iinduced distal hereditary motor neuropathies
17. P54 A dominant negative mutation in FBXO38 is a cause of distal hereditary motor neuropathy (dHMN)
18. P55 Are axonal transport deficits present in a novel mouse model of spinal and bulbar muscular atrophy?
19. P62 Investigating the effects of pharmacological up-regulation of the heat shock response in a transgenic mouse model of inclusion body myopathy
20. O.10 Mutations in a new dynein/dynactin adaptor gene cause Dominant Congenital Spinal Muscular Atrophy (DCSMA) and Hereditary Spastic Paraplegia (HSP)
21. A missense mutation in the mouse TDP-43 gene leads to a gain of TDP-43 mediated splicing function: Implications for neurodegeneration
22. P20 Targeting the endogenous stress response in a mouse model of SBMA
23. P23 Investigating new mutant models of MND
24. P69 Investigating the effects of pharmacological up-regulation of the heat shock response in an in-vitro model of sporadic inclusion body myositis
25. P52 A novel p.glu175x premature stop mutation in the C-terminal end of HSP27 is a cause of CMT2
26. O18 A new mouse model of ALS carrying a point mutation in the mouse Sod1 gene
27. P71 A randomised, double-blinded, placebo-controlled pilot study assessing the safety and tolerability of Arimoclomol in sporadic inclusion body myositis (IBM)
28. P72 The effects of arimoclomol on pathological outcome measures of inclusion body myositis in vitro
29. P16 Investigating novel mutant mouse models of motor neuron disease
30. O08 Characterisation of novel mutations within heat shock protein 27 causing motor axonopathies
31. P13 Investigating pathophysiology and therapeutic strategies in a mouse model of spinal and bulbar muscular atrophy (SBMA)
32. P69 Investigating the effects of pharmacological up-regulation of the heat shock response on protein degradation pathways in an in-vitro model of sporadic inclusion body myositis
33. P7-23 Multiple measures of axonal excitability in a mouse model of Kennedy disease
34. P32 Investigating novel mutant mouse models of motor neuron disease
35. P82 Heat shock protein induction as a therapeutic strategy for inclusion body myositis
36. P80 Augmentation of the heat shock response in an in vitro model of sporadic inclusion body myositis
37. P62 Characterisation of novel mutations within HSP27 causing Charcot–Marie–Tooth disease 2F and distal hereditary motor neuropathy II
38. G.P.4.13 A co-inducer of the heat shock response ameliorates disease in a mouse model of SBMA
39. The effect of peripheral nerve injury on disease progression in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis
40. Inhibition of calpains, by treatment with leupeptin, improves motoneuron survival and muscle function in models of motoneuron degeneration
41. Over-expression of parvalbumin in transgenic mice rescues motoneurons from injury-induced cell death
42. The effect of treatment with BRX-220, a co-inducer of heat shock proteins, on sensory fibers of the rat following peripheral nerve injury
43. Upregulation of Heat Shock Proteins Rescues Motoneurones from Axotomy-Induced Cell Death in Neonatal Rats
44. Inducing Excessive Axonal Growth Results in the Death of Otherwise Healthy Adult Rat Motor Neurones
45. Reducing transmitter release from nerve terminals influences motoneuron survival in developing rats
46. Repeated stimuli for axonal growth causes motoneuron death in adult rats: the effect of botulinum toxin followed by partial denervation
47. Stabilizing neuromuscular contacts reduces motoneuron death caused by paralysis of muscles in neonatal rats
48. Motoneurons innervating partially denervated rat hindlimb muscles remain susceptible to axotomy-induced cell death
49. A simple method for local delivery of various substances to the rat neuromuscular system
50. Induction of transmitter release at the neuromuscular junction prevents motoneuron death after axotomy in neonatal rats
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