Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy.

Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy.

Proximal spinal muscular atrophy (SMA) is a common autosomal recessive childhood form of motor neuron disease. Previous studies have highlighted nerve- and muscle-specific events in SMA, including atrophy of muscle fibres and post-synaptic motor endplates, loss of lower motor neuron cell bodies and...

Szczegółowa specyfikacja

Opis bibliograficzny
Główni autorzy:	Murray, L, Comley, L, Thomson, D, Parkinson, N, Talbot, K, Gillingwater, T
Format:	Journal article
Język:	English
Wydane:	2008

Podobne zapisy

Review: neuromuscular synaptic vulnerability in motor neurone disease: amyotrophic lateral sclerosis and spinal muscular atrophy.
od: Murray, L, i wsp.
Wydane: (2010)

Reversible molecular pathology of skeletal muscle in spinal muscular atrophy.
od: Mutsaers, C, i wsp.
Wydane: (2011)

Neuromuscular junction pathology is correlated with differential motor unit vulnerability in spinal and bulbar muscular atrophy
od: Elana Molotsky, i wsp.
Wydane: (2022-07-01)

Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy.
od: Bäumer, D, i wsp.
Wydane: (2009)

Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy.
od: Karen K Y Ling, i wsp.
Wydane: (2010-11-01)

The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy.
od: Sleigh, J, i wsp.
Wydane: (2011)

Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy.
od: Dirk Bäumer, i wsp.
Wydane: (2009-12-01)

Persistent neuromuscular junction transmission defects in adults with spinal muscular atrophy treated with nusinersen
od: Tristan Weaver, i wsp.
Wydane: (2021-09-01)

The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy
od: James N. Sleigh, i wsp.
Wydane: (2011-07-01)

Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy.
od: Murray, L, i wsp.
Wydane: (2010)

Atrial Septal Defect, Neuromuscular Junction and Skeletal Abnormalities in Spinal Muscular Atrophy Type III
od: Xing-Hua Luan, i wsp.
Wydane: (2017-01-01)

Spinal muscular atrophy.
od: Talbot, K, i wsp.
Wydane: (2001)

Development and Translation of Therapies for Spinal Muscular Atrophy
od: Hannah K. Shorrock, i wsp.
Wydane: (2016-07-01)

Depletion of SMN protein in mesenchymal progenitors impairs the development of bone and neuromuscular junction in spinal muscular atrophy
od: Sang-Hyeon Hann, i wsp.
Wydane: (2024-02-01)

A link between agrin signalling and Cav3.2 at the neuromuscular junction in spinal muscular atrophy
od: Perrine Delers, i wsp.
Wydane: (2022-11-01)

Modeling the Early Phenotype at the Neuromuscular Junction of Spinal Muscular Atrophy Using Patient-Derived iPSCs
od: Michiko Yoshida, i wsp.
Wydane: (2015-04-01)

Therapeutic strategies for spinal muscular atrophy: SMN and beyond
od: Bowerman, M, i wsp.
Wydane: (2017)

Therapeutic strategies for spinal muscular atrophy: SMN and beyond
od: Bowerman, M, i wsp.
Wydane: (2017)

Spinal muscular atrophies reveal motor neuron vulnerability to defects in ribonucleoprotein handling.
od: Anderson, K, i wsp.
Wydane: (2003)

Therapeutic strategies for spinal muscular atrophy: SMN and beyond
od: Melissa Bowerman, i wsp.
Wydane: (2017-08-01)

Chapter 16 Spinal Muscular Atrophy
od: Talbot, K, i wsp.
Wydane: (2003)

SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy.
od: Wishart, T, i wsp.
Wydane: (2010)

Muscle: an independent contributor to the neuromuscular spinal muscular atrophy disease phenotype
od: Narendra N. Jha, i wsp.
Wydane: (2023-09-01)

Cardiac pathology in spinal muscular atrophy: a systematic review
od: C. A. Wijngaarde, i wsp.
Wydane: (2017-04-01)

Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons.
od: Ximena Paez-Colasante, i wsp.
Wydane: (2013-01-01)

Spinal muscular atrophy at the crossroads of basic science and therapy
od: Sleigh, J, i wsp.
Wydane: (2013)

The neuromuscular impact of symptomatic SMN restoration in a mouse model of spinal muscular atrophy
od: W. Arnold, i wsp.
Wydane: (2016-03-01)

Spinal muscular atrophy: Factors that modulate motor neurone vulnerability
od: Wen-Yo Tu, i wsp.
Wydane: (2017-06-01)

“Calcium bombs” as harbingers of synaptic pathology and their mitigation by magnesium at murine neuromuscular junctions
od: Kosala N. Dissanayake, i wsp.
Wydane: (2022-07-01)

Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy
od: Magali Michaud, i wsp.
Wydane: (2010-04-01)

Src inhibition attenuates polyglutamine-mediated neuromuscular degeneration in spinal and bulbar muscular atrophy
od: Madoka Iida, i wsp.
Wydane: (2019-09-01)

Emerging concepts underlying selective neuromuscular dysfunction in infantile-onset spinal muscular atrophy
od: Kishore Gollapalli, i wsp.
Wydane: (2021-01-01)

Spinal Muscular Atrophy
od: J Gordon Millichap
Wydane: (1990-02-01)

Spinal muscular atrophy
od: D'Amico Adele, i wsp.
Wydane: (2011-11-01)

Spinal muscular atrophy at the crossroads of basic science and therapy.
od: Sleigh, J, i wsp.
Wydane: (2013)

ASSESSMENT OF IN VITRO HUMAN NEUROMUSCULAR JUNCTION FUNCTIONALITY USING A CUSTOM MICRO ELECTRODE ARRAY (MEA) PLATFORM FOR THE STUDY OF SPINAL MUSCULAR ATROPHY
od: Pauline Duc, i wsp.
Wydane: (2023-10-01)

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy
od: Turner, B, i wsp.
Wydane: (2008)

Chapter 7 Spinal muscular atrophies and hereditary motor neuropathies.
od: Talbot, K, i wsp.
Wydane: (2007)

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy
od: Ansorge Olaf, i wsp.
Wydane: (2008-10-01)

MOLECULAR ANALYSIS OF CHILDHOOD-ONSET SPINAL MUSCULAR-ATROPHY
od: Theodosiou, A, i wsp.
Wydane: (1995)