Agrin elicits membrane lipid condensation at sites of acetylcholine receptor clusters in C2C12 myotubes.
AuthorsStetzkowski-Marden F, Gaus K, Recouvreur M, Cartaud A, Cartaud J
JournalJ Lipid Res
PubMed ID16816402
'The formation of the neuromuscular junction is characterized by the progressive accumulation of nicotinic acetylcholine receptors (AChRs) in the postsynaptic membrane facing the nerve terminal, induced predominantly through the agrin/muscle-specific kinase (MuSK) signaling cascade. However, the cellular mechanisms linking MuSK activation to AChR clustering are still poorly understood. Here, we ... More
Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice.
AuthorsKong L, Wang X, Choe DW, Polley M, Burnett BG, Bosch-Marcé M, Griffin JW, Rich MM, Sumner CJ,
JournalJ Neurosci
PubMed ID19158308
'The motor neuron disease spinal muscular atrophy (SMA) causes profound muscle weakness that most often leads to early death. At autopsy, SMA is characterized by loss of motor neurons and muscle atrophy, but the initial cellular events that precipitate motor unit dysfunction and loss remain poorly characterized. Here, we examined ... More
Neuromuscular junction integrity after chronic nerve compression injury.
AuthorsMozaffar T, Strandberg E, Abe K, Hilgenberg LG, Smith MA, Gupta R,
JournalJ Orthop Res
PubMed ID18655131
Chronic nerve compression injuries (CNC) are progressive demyelinating disorders characterized by a gradual decline of the nerve conduction velocity (NCV) in the affected nerve region. CNC injury induces a robust Schwann cell response with axonal sprouting, but without morphologic evidence of axonal injury. We hypothesize that early CNC injury occurs ... More
CXCL12α/SDF-1 from perisynaptic Schwann cells promotes regeneration of injured motor axon terminals.
Authors
JournalEMBO Mol Med
PubMed ID28559442
Spinal subpial delivery of AAV9 enables widespread gene silencing and blocks motoneuron degeneration in ALS.
Authors
JournalNat Med
PubMed ID31873312
The neuromuscular junction is a focal point of mTORC1 signaling in sarcopenia.
Authors
JournalNat Commun
PubMed ID32908143
The nuclear envelope protein Net39 is essential for muscle nuclear integrity and chromatin organization.
Authors
JournalNat Commun
PubMed ID33514739
Synaptic Vesicle Precursors and Lysosomes Are Transported by Different Mechanisms in the Axon of Mammalian Neurons.
Authors
JournalCell Rep
PubMed ID32553155
Sonic Hedgehog repression underlies gigaxonin mutation-induced motor deficits in giant axonal neuropathy.