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Comment
. 2014 Nov;85(11):1288-9.
doi: 10.1136/jnnp-2014-308288. Epub 2014 Jun 26.

A zebrafish model exemplifies the long preclinical period of motor neuron disease

Affiliations
Comment

A zebrafish model exemplifies the long preclinical period of motor neuron disease

Tennore M Ramesh et al. J Neurol Neurosurg Psychiatry. 2014 Nov.
No abstract available

Keywords: ALS; Clinical Neurology; Genetics; Motor Neuron Disease; Neurobiology.

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Figures

Figure 1
Figure 1
Chronic preclinical changes in sod1 zebrafish model of ALS. As early as 24 hpf (embryos), the zebrafish embryos show neuronal stress predominantly in the inhibitory interneurons of the spinal cord. By 96 hpf (larvae), a reduction in inhibitory input to motor neurons is observed, suggesting lack of sufficient innervation by inhibitory interneurons on the motor neurons and/or denervation of existing inhibitory synapses on motor neurons. Interestingly, the motor neurons do not show neuronal stress at this time. However, over a span of a few months (adults), motor neurons in the adult spinal cord show neuronal stress and the synapses of stressed motor neurons show clear abnormalities at the neuromuscular junction (NMJ). Thus, in the mutant sod1 zebrafish model, gradual and incremental changes occur within the neural circuit to finally impact on motor neurons and their accompanying neuromuscular connectivity. hpf, hours postfertilisation.

Comment in

  • Response to a letter by Dr T Ramesh.
    Eisen A. Eisen A. J Neurol Neurosurg Psychiatry. 2014 Nov;85(11):1289. doi: 10.1136/jnnp-2014-308588. Epub 2014 Jun 26. J Neurol Neurosurg Psychiatry. 2014. PMID: 24970904 No abstract available.

Comment on

References

    1. Eisen A, Kiernan M, Mitsumoto H, et al. Amyotrophic lateral sclerosis: a long preclinical period? J Neurol Neurosurg Psychiatry. 2014;85:1232–8. - PubMed
    1. Ramesh T, Lyon AN, Pineda RH, et al. A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron disease. Dis Model Mech 2010;3:652–62. - PMC - PubMed
    1. McGown A, McDearmid JR, Panagiotaki N, et al. Early interneuron dysfunction in ALS: insights from a mutant sod1 zebrafish model. Ann Neurol 2013;73:246–58. - PMC - PubMed
    1. Batulan Z, Shinder GA, Minotti S, et al. High threshold for induction of the stress response in motor neurons is associated with failure to activate HSF1. J Neurosci 2003;23:5789–98. - PMC - PubMed