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Peer-reviewedCC BYFrontiers in molecular neuroscience

SH3BP2 deficiency in skeletal muscle leads to neuromuscular junction abnormalities and decreased muscle strength.

  • Bhola Shankar Pradhan1,2
  • Krzysztof M Bernadzki2
  • Yauhen Bandaruk1
  • Anna Siudzińska1
  • Teresa De Cicco1
  • Małgorzata Sotomska1
  • Marta Gawor2
  • Margareta Jabłońska1
  • Joanna Krzemień2
  • Grzegorz Chodaczek1
  • Said Hashemolhosseini3
  • Tomasz J Prószyński1,2
  1. 1Łukasiewicz Research Network-PORT Polish Center for Technology Development, Wrocław, Poland.
  2. 2Nencki Institute of Experimental Biology, Polish Academy of Sciences, Warsaw, Poland.
  3. 3Institute of Biochemistry, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
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Abstract

Assembly and stabilization of a high density of acetylcholine receptors (AChRs) at the neuromuscular junction postsynaptic domain is fundamental for proper synaptic transmission. Here, we report the identification of a scaffold protein, SH3BP2, that exhibits polyvalent and direct interactions with the dystrophin-glycoprotein complex (DGC) and AChR pentamers. Loss-of-function experiments in cultured myotubes revealed that SH3BP2 promotes formation of AChR assemblies in muscle cells. Similarly, muscle-specific deletion of SH3BP2 in mice impairs AChR clustering during embryonic development and causes multiple neuromuscular junction morphological and ultrastructural abnormalities in adult animals. Mutant mice also exhibit defects in synaptic transmission, muscle strength, and general fitness. Our study identifies a novel role for SH3BP2 in modulating the neuromuscular junction in skeletal muscles.

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Cite

BibTeXRIS
BibTeX
@article{pradhan2026sh3bp2,
  title = {{SH3BP2 deficiency in skeletal muscle leads to neuromuscular junction abnormalities and decreased muscle strength.}},
  author = {Bhola Shankar Pradhan and Krzysztof M Bernadzki and Yauhen Bandaruk and Anna Siudzińska and Teresa De Cicco and Małgorzata Sotomska and Marta Gawor and Margareta Jabłońska and Joanna Krzemień and Grzegorz Chodaczek and Said Hashemolhosseini and Tomasz J Prószyński},
  journal = {Frontiers in molecular neuroscience},
  year = {2026},
  month = oct,
  doi = {10.3389/fnmol.2026.1985823},
  url = {https://europepmc.org/article/MED/42898440},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - SH3BP2 deficiency in skeletal muscle leads to neuromuscular junction abnormalities and decreased muscle strength.
AU  - Bhola Shankar Pradhan
AU  - Krzysztof M Bernadzki
AU  - Yauhen Bandaruk
AU  - Anna Siudzińska
AU  - Teresa De Cicco
AU  - Małgorzata Sotomska
AU  - Marta Gawor
AU  - Margareta Jabłońska
AU  - Joanna Krzemień
AU  - Grzegorz Chodaczek
AU  - Said Hashemolhosseini
AU  - Tomasz J Prószyński
PY  - 2026
DA  - 2026/10/05
JO  - Frontiers in molecular neuroscience
DO  - 10.3389/fnmol.2026.1985823
UR  - https://europepmc.org/article/MED/42898440
AB  - Assembly and stabilization of a high density of acetylcholine receptors (AChRs) at the neuromuscular junction postsynaptic domain is fundamental for proper synaptic transmission. Here, we report the identification of a scaffold protein, SH3BP2, that exhibits polyvalent and direct interactions with the dystrophin-glycoprotein complex (DGC) and AChR pentamers. Loss-of-function experiments in cultured myotubes revealed that SH3BP2 promotes formation of AChR assemblies in muscle cells. Similarly, muscle-specific deletion of SH3BP2 in mice impairs AChR clustering during embryonic development and causes multiple neuromuscular junction morphological and ultrastructural abnormalities in adult animals. Mutant mice also exhibit defects in synaptic transmission, muscle strength, and general fitness. Our study identifies a novel role for SH3BP2 in modulating the neuromuscular junction in skeletal muscles.
SN  - 1662-5099
ER  -