University of Sussex
Browse

File(s) not publicly available

Mice, the motor system, and human motor neuron pathology

journal contribution
posted on 2023-06-07, 21:27 authored by S J Nicholson, A S Witherden, Majid HafezparastMajid Hafezparast, J E Martin, E M Fisher
Motor neurons are among some of the most unusual cells in the body becaue of their immense size and their role as the critical link between the motor centers of the brain and the muscles. In addition to their intrinsic biological interest, it is vital that we gain a better understanding of these cells and their pathology, since motor neuron degenerative diseases are lethal disorders that affect young and old and are relatively common. For example, one form of spinal muscular atrophy (SMA) is the most common genetic killer of children in the developed world. Amyotrophic lateral sclerosis (ALS), another form of motor neuron degeneration, is the third most common neurodegenerative cause of adult death, after Alzheimer's disease and Parkinson's disease, and is significantly more common than multiple sclerosis (Motor Neurone Disease Association 1998). Currently, approximately 1 in 500 people in England and Wares who die have a form of motor neuron disease (Motor Neurone Disease Association 1998). Each year, 5000 Americans are diagnosed with ALS, and of these, 10% are under 40 years old. Mouse models of motor neuron degeneration are essential for understanding the causes and mechanisms of motor neuron pathology. These mice are yielding important information that will ultimately lead to treatments and potentially cures for these diseases.

History

Publication status

  • Published

Journal

Mammalian Genome

ISSN

09388990

Publisher

Springer Verlag

Issue

12

Volume

11

Page range

1041-1052

Department affiliated with

  • Neuroscience Publications

Full text available

  • No

Peer reviewed?

  • No

Legacy Posted Date

2012-02-06

Usage metrics

    University of Sussex (Publications)

    Categories

    No categories selected

    Exports

    RefWorks
    BibTeX
    Ref. manager
    Endnote
    DataCite
    NLM
    DC