Type

Journal Article

Authors

Kay Ohlendieck
Martin Clynes
Michael Henry
Paula Meleady
Paul Dowling
Thomas Schmitt-John
Ashling Holland

Subjects

Biochemistry

Topics
muscle proteins contractile proteins biology myosin light chains motor neuron disease mass spectrometry adaptation physiological muscle skeletal protein binding

Intricate effects of primary motor neuronopathy on contractile proteins and metabolic muscle enzymes as revealed by label-free mass spectrometry. (2014)

Abstract While the long-term physiological adaptation of the neuromuscular system to changed functional demands is usually reflected by unilateral skeletal muscle transitions, the progressive degeneration of distinct motor neuron populations is often associated with more complex changes in the abundance and/or isoform expression pattern of contractile proteins and metabolic enzymes. In order to evaluate these intricate effects of primary motor neuronopathy on the skeletal muscle proteome, label-free MS was employed to study global alterations in the WR (wobbler) mouse model of progressive neurodegeneration. In motor neuron disease, fibre-type specification and the metabolic weighting of bioenergetic pathways appear to be strongly influenced by both a differing degree of a subtype-specific vulnerability of neuromuscular synapses and compensatory mechanisms of fibre-type shifting. Proteomic profiling confirmed this pathobiochemical complexity of disease-induced changes and showed distinct alterations in 72 protein species, including a variety of fibre-type-specific isoforms of contractile proteins, metabolic enzymes, metabolite transporters and ion-regulatory proteins, as well as changes in molecular chaperones and various structural proteins. Increases in slow myosin light chains and the troponin complex and a decrease in fast MBP (myosin-binding protein) probably reflect the initial preferential loss of the fast type of neuromuscular synapses in motor neuron disease.
Collections Ireland -> Maynooth University -> Academic Unit = Faculty of Science and Engineering: Biology
Ireland -> Maynooth University -> Type = Article
Ireland -> Dublin City University -> PubMed
Ireland -> Maynooth University -> Academic Unit = Faculty of Science and Engineering
Ireland -> Maynooth University -> Status = Published
Ireland -> Maynooth University -> Open Access DRIVERset

Full list of authors on original publication

Kay Ohlendieck, Martin Clynes, Michael Henry, Paula Meleady, Paul Dowling, Thomas Schmitt-John, Ashling Holland

Experts in our system

1
Kay Ohlendieck
Maynooth University
Total Publications: 119
 
2
Martin Clynes
Dublin City University
Total Publications: 209
 
3
Michael Henry
Dublin City University
Total Publications: 78
 
4
Paula Meleady
Dublin City University
Total Publications: 95
 
5
Paul Dowling
Dublin City University
Total Publications: 79
 
6
Ashling Holland
Maynooth University
Total Publications: 9