IMR Press / FBS / Volume 4 / Issue 4 / DOI: 10.2741/S351

Frontiers in Bioscience-Scholar (FBS) is published by IMR Press from Volume 13 Issue 1 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.


Unaffected motor endplate occupancy in eye muscles of ALS G93A mouse model

Show Less
1 Department of Integrative Medical Biology, Section for Anatomy, Umea University, SE-901 87 Umea, Sweden
2 Department of Medical Biosciences, Section for Pathology, Umea University, SE-901 85 Umea, Sweden
3 Department of Clinical Sciences, Ophthalmology Umea University, SE-901 87 Umea, Sweden

*Author to whom correspondence should be addressed.

Academic Editor: Homa Tajsharghi

Front. Biosci. (Schol Ed) 2012, 4(4), 1547–1555;
Published: 1 June 2012
(This article belongs to the Special Issue Skeletal muscle diseases)

Amyotrophic lateral sclerosis (ALS) is a progressive, lethal neurodegenerative disorder characterised by selective loss of motor neurons with accompanying muscle paralysis and respiratory failure. Despite progressive paralysis in trunk and extremity muscles, disturbed eye motility is not a hallmark of ALS. Extraocular muscles (EOMs) of terminal ALS patients show far less morphological signs of disease than their limb muscles. One of the earliest signs of the disease in the transgenic G93A SOD1 mouse model of ALS is loss of motor neuron contact at the neuromuscular junctions (NMJ) in limb muscles. We used immunohistochemistry to identify NMJs and evaluate innervation in EOMs and limb muscles of G93A mice. In G93A limb muscles, loss of axonal contact was seen in 6-82% of the NMJs. On the contrary, the degree of endplate occupancy in the EOMs did not differ between transgenic mice and wild-type controls. We propose that EOM-specific properties make these muscles more resistant to the underlying pathophysiological process of ALS and that the EOMs are a useful model to advance our understanding of ALS.

Back to top