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Article

Passive Transfer of Blood Sera from ALS Patients with Identified Mutations Results in Elevated Motoneuronal Calcium Level and Loss of Motor Neurons in the Spinal Cord of Mice

1
Biological Research Centre, Institute of Biophysics, 62 Temesvári krt., 6726 Szeged, Hungary
2
Theoretical Medicine Doctoral School, University of Szeged, 97 Tisza Lajos krt., 6722 Szeged, Hungary
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Albert Szent-Györgyi Health Centre, Department of Neurology, University of Szeged, 6 Semmelweis u., 6725 Szeged, Hungary
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Department of Medical Genetics, University of Szeged, 4/B Szőkefalvi-Nagy Béla u., 6720 Szeged, Hungary
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Dermatological Research Group, Hungarian Academy of Sciences, University of Szeged, 4/B Szőkefalvi-Nagy Béla u., 6720 Szeged, Hungary
6
Department of Neurology, Aalborg University Hospital, 15 Skovvej Sdr., 9000 Aalborg, Denmark
*
Authors to whom correspondence should be addressed.
Academic Editor: Demetrios A. Arvanitis
Int. J. Mol. Sci. 2021, 22(18), 9994; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22189994
Received: 13 August 2021 / Revised: 9 September 2021 / Accepted: 13 September 2021 / Published: 16 September 2021
(This article belongs to the Special Issue Calcium Handling)
Introduction: Previously, we demonstrated the degeneration of axon terminals in mice after repeated injections of blood sera from amyotrophic lateral sclerosis (ALS) patients with identified mutations. However, whether a similar treatment affects the cell body of motor neurons (MNs) remained unresolved. Methods: Sera from healthy individuals or ALS patients with a mutation in different ALS-related genes were intraperitoneally injected into ten-week-old male Balb/c mice (n = 3/serum) for two days. Afterward, the perikaryal calcium level was measured using electron microscopy. Furthermore, the optical disector method was used to evaluate the number of lumbar MNs. Results: The cytoplasmic calcium level of the lumbar MNs of the ALS-serum-treated mice, compared to untreated and healthy-serum-treated controls, was significantly elevated. While injections of the healthy serum did not reduce the number of MNs compared to the untreated control group, ALS sera induced a remarkable loss of MNs. Discussion: Similarly to the distant motor axon terminals, the injection of blood sera of ALS patients has a rapid degenerative effect on MNs. Analogously, the magnitude of the evoked changes was specific to the type of mutation; furthermore, the degeneration was most pronounced in the group treated with sera from ALS patients with a mutation in the chromosome 9 open reading frame 72 gene. View Full-Text
Keywords: amyotrophic lateral sclerosis; passive transfer; blood serum; motoneuronal calcium increase; motoneuronal loss; C9ORF72 amyotrophic lateral sclerosis; passive transfer; blood serum; motoneuronal calcium increase; motoneuronal loss; C9ORF72
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MDPI and ACS Style

Polgár, T.F.; Meszlényi, V.; Nógrádi, B.; Körmöczy, L.; Spisák, K.; Tripolszki, K.; Széll, M.; Obál, I.; Engelhardt, J.I.; Siklós, L.; Patai, R. Passive Transfer of Blood Sera from ALS Patients with Identified Mutations Results in Elevated Motoneuronal Calcium Level and Loss of Motor Neurons in the Spinal Cord of Mice. Int. J. Mol. Sci. 2021, 22, 9994. https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22189994

AMA Style

Polgár TF, Meszlényi V, Nógrádi B, Körmöczy L, Spisák K, Tripolszki K, Széll M, Obál I, Engelhardt JI, Siklós L, Patai R. Passive Transfer of Blood Sera from ALS Patients with Identified Mutations Results in Elevated Motoneuronal Calcium Level and Loss of Motor Neurons in the Spinal Cord of Mice. International Journal of Molecular Sciences. 2021; 22(18):9994. https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22189994

Chicago/Turabian Style

Polgár, Tamás F., Valéria Meszlényi, Bernát Nógrádi, Laura Körmöczy, Krisztina Spisák, Kornélia Tripolszki, Márta Széll, Izabella Obál, József I. Engelhardt, László Siklós, and Roland Patai. 2021. "Passive Transfer of Blood Sera from ALS Patients with Identified Mutations Results in Elevated Motoneuronal Calcium Level and Loss of Motor Neurons in the Spinal Cord of Mice" International Journal of Molecular Sciences 22, no. 18: 9994. https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22189994

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