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Article

Experimental Motor Neuron Disease Induced in Mice with Long-Term Repeated Intraperitoneal Injections of Serum from ALS Patients

1
Department of Neurology, University of Szeged, H-6725 Szeged, Hungary
2
Department of Neurology, Aalborg University Hospital, DK-9000 Aalborg, Denmark
3
Foundation for the Future of Biomedical Sciences in Szeged, Szeged Scientist Academy, H-6721 Szeged, Hungary
4
Institute of Biophysics, Biological Research Centre of the Hungarian Academy of Sciences, H-6726 Szeged, Hungary
5
Clinical Institute of Neurology, Medical University of Vienna, Vienna A-1090, Austria
6
Department of Medical Genetics, University of Szeged, H-6720 Szeged, Hungary
7
Hungarian Academy of Sciences – University of Szeged, Dermatological Research Group, H-6720 Szeged, Hungary
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Int. J. Mol. Sci. 2019, 20(10), 2573; https://doi.org/10.3390/ijms20102573
Received: 22 March 2019 / Revised: 2 May 2019 / Accepted: 23 May 2019 / Published: 25 May 2019
(This article belongs to the Special Issue Amyotrophic Lateral Sclerosis: Highlights of Its Complexity)
In an earlier study, signs of commencing degeneration of spinal motor neurons were induced in mice with short-term intraperitoneal injections of immunoglobulin G (IgG) taken from patients with amyotrophic lateral sclerosis (ALS). Since in that study, neither weakness nor loss of motor neurons was noted, to test whether the ALS IgG in this paradigm has the potential to evoke relentless degeneration of motor neurons, treatment with repeated injections over a longer period was carried out. Mice were systematically injected intraperitoneally with serum taken from ALS patients over a 75-day period. At selected time points, the isometric force of the limbs, number of spinal motor neurons and their intracellular calcium levels were determined. Furthermore, markers of glial activation and the motoneuronal uptake of human IgG were monitored. During this period, gliosis and progressive motoneuronal degeneration developed, which led to gradual loss of spinal motor neurons, more than 40% at day 21, along with decreasing muscle strength in the limbs. The inclusion-like accumulation of IgG appeared in the perikarya with the increase of intracellular calcium in the cell bodies and motor nerve terminals. Our results demonstrate that ALS serum can transfer motor neuron disease to mice. View Full-Text
Keywords: ALS; motor neuron; degeneration; serum; passive transfer; IgG uptake; intracellular calcium ALS; motor neuron; degeneration; serum; passive transfer; IgG uptake; intracellular calcium
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MDPI and ACS Style

Obál, I.; Nógrádi, B.; Meszlényi, V.; Patai, R.; Ricken, G.; Kovacs, G.G.; Tripolszki, K.; Széll, M.; Siklós, L.; Engelhardt, J.I. Experimental Motor Neuron Disease Induced in Mice with Long-Term Repeated Intraperitoneal Injections of Serum from ALS Patients. Int. J. Mol. Sci. 2019, 20, 2573. https://doi.org/10.3390/ijms20102573

AMA Style

Obál I, Nógrádi B, Meszlényi V, Patai R, Ricken G, Kovacs GG, Tripolszki K, Széll M, Siklós L, Engelhardt JI. Experimental Motor Neuron Disease Induced in Mice with Long-Term Repeated Intraperitoneal Injections of Serum from ALS Patients. International Journal of Molecular Sciences. 2019; 20(10):2573. https://doi.org/10.3390/ijms20102573

Chicago/Turabian Style

Obál, Izabella, Bernát Nógrádi, Valéria Meszlényi, Roland Patai, Gerda Ricken, Gabor G. Kovacs, Kornélia Tripolszki, Márta Széll, László Siklós, and József I. Engelhardt 2019. "Experimental Motor Neuron Disease Induced in Mice with Long-Term Repeated Intraperitoneal Injections of Serum from ALS Patients" International Journal of Molecular Sciences 20, no. 10: 2573. https://doi.org/10.3390/ijms20102573

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