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- Publications
- Influence
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis
- Laura Ferraiuolo, J. Kirby, A. Grierson, M. Sendtner, P. Shaw
- Biology, Medicine
- Nature Reviews Neurology
- 1 November 2011
Amyotrophic lateral sclerosis (ALS) is a genetically diverse disease. At least 15 ALS-associated gene loci have so far been identified, and the causative gene is known in approximately 30% of… Expand
Microglia Induce Motor Neuron Death via the Classical NF-κB Pathway in Amyotrophic Lateral Sclerosis
- Ashley E. Frakes, Laura Ferraiuolo, +10 authors B. Kaspar
- Biology, Medicine
- Neuron
- 5 March 2014
Neuroinflammation is one of the most striking hallmarks of amyotrophic lateral sclerosis (ALS). Nuclear factor-kappa B (NF-κB), a master regulator of inflammation, is upregulated in spinal cords of… Expand
Microarray Analysis of the Cellular Pathways Involved in the Adaptation to and Progression of Motor Neuron Injury in the SOD1 G93A Mouse Model of Familial ALS
- Laura Ferraiuolo, P. Heath, H. Holden, P. Kasher, J. Kirby, P. Shaw
- Biology, Medicine
- The Journal of Neuroscience
- 22 August 2007
The cellular pathways of motor neuronal injury have been investigated in the SOD1 G93A murine model of familial amyotrophic lateral sclerosis (ALS) using laser-capture microdissection and microarray… Expand
The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy
- C. P. Webster, E. F. Smith, +12 authors Kurt J. De Vos
- Biology, Medicine
- The EMBO journal
- 22 June 2016
A GGGGCC hexanucleotide repeat expansion in the C9orf72 gene is the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD). C9orf72 encodes two C9orf72… Expand
Mutations in CHMP2B in Lower Motor Neuron Predominant Amyotrophic Lateral Sclerosis (ALS)
- L. Cox, Laura Ferraiuolo, +13 authors P. Shaw
- Biology, Medicine
- PloS one
- 24 March 2010
Background Amyotrophic lateral sclerosis (ALS), a common late-onset neurodegenerative disease, is associated with fronto-temporal dementia (FTD) in 3–10% of patients. A mutation in CHMP2B was… Expand
Improving single injection CSF delivery of AAV9-mediated gene therapy for SMA: a dose-response study in mice and nonhuman primates.
- K. Meyer, Laura Ferraiuolo, +11 authors B. Kaspar
- Biology, Medicine
- Molecular therapy : the journal of the American…
- 1 March 2015
Spinal muscular atrophy (SMA) is the most frequent lethal genetic neurodegenerative disorder in infants. The disease is caused by low abundance of the survival of motor neuron (SMN) protein leading… Expand
Phosphatase and tensin homologue/protein kinase B pathway linked to motor neuron survival in human superoxide dismutase 1-related amyotrophic lateral sclerosis
- J. Kirby, Ke Ning, +10 authors M. Azzouz
- Biology, Medicine
- Brain : a journal of neurology
- 12 January 2011
Gene expression profiling has been used previously with spinal cord homogenates and laser capture microdissected motor neurons to determine the mechanisms involved in neurodegeneration in amyotrophic… Expand
Gene expression profiling in human neurodegenerative disease
- J. Cooper-Knock, J. Kirby, Laura Ferraiuolo, P. Heath, M. Rattray, P. J. Shaw
- Biology, Medicine
- Nature Reviews Neurology
- 1 September 2012
Transcriptome study in neurodegenerative disease has advanced considerably in the past 5 years. Increasing scientific rigour and improved analytical tools have led to more-reproducible data. Many… Expand
Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALS.
- K. Foust, D. Salazar, +8 authors B. Kaspar
- Biology, Medicine
- Molecular therapy : the journal of the American…
- 1 December 2013
Mutations in superoxide dismutase 1 (SOD1) are linked to familial amyotrophic lateral sclerosis (ALS) resulting in progressive motor neuron death through one or more acquired toxicities. Involvement… Expand
Dysregulation of astrocyte-motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosis.
- Laura Ferraiuolo, A. Higginbottom, +4 authors P. Shaw
- Biology, Medicine
- Brain : a journal of neurology
- 1 September 2011
Amyotrophic lateral sclerosis is a neurodegenerative disease in which death of motoneurons leads to progressive failure of the neuromuscular system resulting in death frequently within 2-3 years of… Expand