Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the death of upper and lower motor neurons. Numerous studies show that structural and functional impairments of neuromuscular junctions (NMJ) occur as early as the presymptomatic stage of ALS. NMJ involv...
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| Language: | English |
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Research Center of Neurology
2025-04-01
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| Series: | Анналы клинической и экспериментальной неврологии |
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| Online Access: | https://annaly-nevrologii.com/pathID/article/viewFile/1070/pdf |
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| author | Aydar N. Khabibrakhmanov Liaisan A. Akhmadieva Kerim K. Nagiev Marat A. Mukhamedyarov |
| author_facet | Aydar N. Khabibrakhmanov Liaisan A. Akhmadieva Kerim K. Nagiev Marat A. Mukhamedyarov |
| author_sort | Aydar N. Khabibrakhmanov |
| collection | DOAJ |
| description | Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the death of upper and lower motor neurons. Numerous studies show that structural and functional impairments of neuromuscular junctions (NMJ) occur as early as the presymptomatic stage of ALS. NMJ involvement is independent and one of the primary events in ALS pathogenesis. Aim: to review the data on characteristics and mechanisms of NMJ dysfunction at pre- and postsynaptic levels in ALS patients and a transgenic animal model of the disease. Furthermore, we report on the dysfunction of perisynaptic Schwann cells and impaired mechanisms of motor neuron and skeletal muscle interaction in ALS, with a focus on reviewed publications on targeting of molecular mechanisms underlying NMJ dysfunction and disruption in ALS. The NMJ may be a potential target for novel therapeutic approaches for ALS. |
| format | Article |
| id | doaj-art-df492f7c65d34c57907296597f72ecbb |
| institution | OA Journals |
| issn | 2075-5473 2409-2533 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Research Center of Neurology |
| record_format | Article |
| series | Анналы клинической и экспериментальной неврологии |
| spelling | doaj-art-df492f7c65d34c57907296597f72ecbb2025-08-20T02:20:10ZengResearch Center of NeurologyАнналы клинической и экспериментальной неврологии2075-54732409-25332025-04-01191536110.17816/ACEN.1070721Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosisAydar N. Khabibrakhmanov0https://orcid.org/0000-0001-5625-8658Liaisan A. Akhmadieva1https://orcid.org/0009-0000-4926-3192Kerim K. Nagiev2https://orcid.org/0009-0000-1577-9780Marat A. Mukhamedyarov3https://orcid.org/0000-0002-0397-9002Kazan State Medical UniversityKazan State Medical UniversityKazan State Medical UniversityKazan State Medical UniversityAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the death of upper and lower motor neurons. Numerous studies show that structural and functional impairments of neuromuscular junctions (NMJ) occur as early as the presymptomatic stage of ALS. NMJ involvement is independent and one of the primary events in ALS pathogenesis. Aim: to review the data on characteristics and mechanisms of NMJ dysfunction at pre- and postsynaptic levels in ALS patients and a transgenic animal model of the disease. Furthermore, we report on the dysfunction of perisynaptic Schwann cells and impaired mechanisms of motor neuron and skeletal muscle interaction in ALS, with a focus on reviewed publications on targeting of molecular mechanisms underlying NMJ dysfunction and disruption in ALS. The NMJ may be a potential target for novel therapeutic approaches for ALS.https://annaly-nevrologii.com/pathID/article/viewFile/1070/pdfamyotrophic lateral sclerosisneuromuscular junctionnerve terminalskeletal musclemotor unit |
| spellingShingle | Aydar N. Khabibrakhmanov Liaisan A. Akhmadieva Kerim K. Nagiev Marat A. Mukhamedyarov Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis Анналы клинической и экспериментальной неврологии amyotrophic lateral sclerosis neuromuscular junction nerve terminal skeletal muscle motor unit |
| title | Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis |
| title_full | Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis |
| title_fullStr | Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis |
| title_full_unstemmed | Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis |
| title_short | Mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis |
| title_sort | mechanisms of neuromuscular junction dysfunction in amyotrophic lateral sclerosis |
| topic | amyotrophic lateral sclerosis neuromuscular junction nerve terminal skeletal muscle motor unit |
| url | https://annaly-nevrologii.com/pathID/article/viewFile/1070/pdf |
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