Selective loss of alpha motor neurons with sparing of gamma motor neurons and spinal cord cholinergic neurons in a mouse model of spinal muscular atrophy

Autor: Rachael A. Powis, Thomas H. Gillingwater

Publikováno v: Powis, R A & Gillingwater, T 2016, ' Selective loss of alpha motor neurons with sparing of gamma motor neurons and spinal cord cholinergic neurons in a mouse model of spinal muscular atrophy ', Journal of Anatomy, vol. 228, no. 3 . https://doi.org/10.1111/joa.12419

Spinal muscular atrophy (SMA) is a neuromuscular disease characterised primarily by loss of lower motor neurons from the ventral grey horn of the spinal cord and proximal muscle atrophy. Recent experiments utilising mouse models of SMA have demonstra

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d31e725a3e46d94b1e11d29cb4db9180
https://doi.org/10.1111/joa.12419

Correction: Corrigendum: Survival Motor Neuron (SMN) protein is required for normal mouse liver development

Autor: Eva Szunyogova, Francesco Muntoni, Rachael A. Powis, Gillian K. Maxwell, Haiyan Zhou, Simon H. Parson, Thomas H. Gillingwater

Publikováno v: Scientific Reports. 6

Scientific Reports 6: Article number: 34635; published online: 04 October 2016; updated: 10 November 2016.

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::2dad1d199e7134c76a10db1f561a794d
https://doi.org/10.1038/srep35898

Survival of motor neurone protein is required for normal postnatal development of the spleen

Autor: Simon H. Parson, Hannah K. Shorrock, Thomas H. Gillingwater, Alison K. Thomson, Rachael A. Powis, Kathryn J. Swoboda, Eilidh Somers, Kelley J. Murphy

Publikováno v: Thomson, A, Somers, E, Powis, R, Shorrock, H K, Murphy, K, Swoboda, K J, Gillingwater, T & Parson, S H 2016, ' Survival of motor neurone protein is required for normal postnatal development of the spleen ', Journal of Anatomy . https://doi.org/10.1111/joa.12546

Spinal muscular atrophy (SMA), traditionally described as a predominantly childhood form of motor neurone disease, is the leading genetic cause of infant mortality. Although motor neurones are undoubtedly the primary affected cell type, the severe in

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ba2056ed27ed09345adeb468d6002f01
https://hdl.handle.net/20.500.11820/2e1a6a31-a480-4112-8139-fdadb62c2eba

Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy

Autor: J. Robin Highley, Paul R. Heath, Nicholas M. Morton, Thomas Becker, Ewout J N Groen, Derek Thomson, Thomas M. Wishart, Rachael A. Powis, Penelope J Boyd, Catherina G. Becker, Roderick N. Carter, Wen-Yo Tu, Thomas H. Gillingwater, Anna A L Motyl, Sophie R. Thomson, Hannah K. Shorrock, Laura C. Graham

Publikováno v: PLoS Genetics, Vol 13, Iss 4, p e1006744 (2017)
Boyd, P J, Tu, W-Y, Shorrock, H K, Groen, E J N, Carter, R N, Powis, R A, Thomson, S R, Thomson, D, Graham, L C, Motyl, A A L, Wishart, T M, Highley, J R, Morton, N M, Becker, T, Becker, C G, Heath, P R & Gillingwater, T H 2017, ' Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy ', PLoS Genetics, vol. 13, no. 4, e1006744 . https://doi.org/10.1371/journal.pgen.1006744
PLoS Genetics

Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6ea50dbb34fd8d58066abee13b6d717f
https://doi.org/10.1371/journal.pgen.1006744