ALS mutations in both human skeletal muscle and motoneurons differentially affects neuromuscular junction integrity and function

Autor:	Agnes Badu-Mensah, Xiufang Guo, Siddharth Nimbalkar, Yunqing Cai, James J. Hickman
Rok vydání:	2022
Předmět:	Biomaterials Motor Neurons Superoxide Dismutase-1 Mechanics of Materials Amyotrophic Lateral Sclerosis Mutation Biophysics Ceramics and Composites Neuromuscular Junction Humans Bioengineering Receptors Cholinergic Muscle Skeletal
Zdroj:	Biomaterials. 289
ISSN:	1878-5905
Popis:	There is evidence for the involvement of human skeletal muscle (hSKM) in ALS neuromuscular junction (NMJ) dysfunction. However, the specific avenue by which the hSKM contributes to NMJ disruption is not well understood due to limited human-based studies performed to investigate the subject. Thus, hSKM and human motoneurons (hMN) generated from induced pluripotent stem cells of healthy individuals (WT) and ALS patients with two different SOD1 mutations were integrated into functional NMJ systems to investigate and compare the pathological contribution of the hSKM and hMN to ALS NMJ disruption. Morphological assessment of ALS NMJs demonstrated reduced acetylcholine receptor clustering in the post-synaptic membrane of co-cultures with ALS hSKM (hSKM
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5a809d93e707355a8fdf0b3aaf4c72e9 https://pubmed.ncbi.nlm.nih.gov/36084484 Zobrazit plný text záznamu Full Text from ScienceDirect