The Composition, Development, and Regeneration of Neuromuscular Junctions.
The Composition, Development, and Regeneration of Neuromuscular Junctions. Curr Top Dev Biol. 2018;126:99-124 Authors: Liu W, Chakkalakal JV Abstract The neuromuscular junction (NMJ) is the specialized site that connects the terminal of a motor neuron axon to skeletal muscle. As a synapse NMJ integrity is essential for transducing motor neuron signals that initiate skeletal muscle contraction. Many diseases and skeletal muscle aging are linked to impaired NMJ function and the associated muscle wasting. In this chapter we review the components of an NMJ and, the processes of NMJ development, maturation, and regeneration. Also, we briefly discuss the cellular and molecular mechanisms of NMJ decline in the context of disease and aging. PMID: 29305005 [PubMed - in process]
This article has been withdrawn by the authors. The Journal questioned Figs. 2A and 7A. The original data and originally submitted figures were not available for evaluation. The authors stand by the reproducibility of the experimental data and the conclusions of the paper. The paper, with confirmatory data supporting the results, can be obtained by contacting the authors.
Conclusion: RA-induced differentiation in neurons could exert a suppressive effect on the activity of IKCa channels.Cell Physiol Biochem 2018;48:2374 –2388
CONCLUSIONS: Plastin 3 overexpression in NSC-34 cells did not elicit an antioxidative effect following serum deprivation. PMID: 30106460 [PubMed - as supplied by publisher]
PMID: 30105827 [PubMed - as supplied by publisher]
We examined biceps and end plate morphologies and monitored selected factors involved in end plate function. Structural volumes were determined from 3D reconstructions that were generated for the end plates. Wobbler mice exhibited size reductions of both the muscle fibers and the end plates within the biceps, and we found that the end plate volumes were the most sensitive indicator of the degeneration. Concurrently, we found increases in calcitonin gene-related peptide (CGRP) and its receptor in wobbler biceps and spinal cord. We also found increases in gene expression of two acetylcholine receptors within the wobbler bice...
ConclusionIntegrated PET-MR imaging demonstrates associations between markers for neuronal integrity and neuroinflammation and may provide valuable insights into disease mechanisms in ALS.
Spasticity can be a complicating symptom of a variety of neurological conditions such as multiple sclerosis, motor neuron disease, Creutzfeldt-Jakob disease, or post-stroke. Several pharmacological treatment options are available, including baclofen, tizanidine, gabapentin, botulinum toxin A and tetrahydrocannabinol/canabidiol (1, 2). However, treatment feasibility decreases as the end of life approaches, e.g. when patients are no longer able to tolerate oral medications, the onset of action would be too long, or the initiation of parenteral drug therapy (intravenous, intrathecal) is no longer indicated or appropriate.
ina Dolei The human endogenous retrovirus (HERV)-K, human mouse mammary tumor virus like-2 (HML-2) subgroup of HERVs is activated in several tumors and has been related to prostate cancer progression and motor neuron diseases. The cellular splicing factor 2/alternative splicing factor (SF2/ASF) is a positive regulator of gene expression, coded by a potent proto-oncogene, amplified, and abnormally expressed in tumors. TAR DNA-binding protein-43 (TDP-43) is a DNA/RNA-binding protein, negative regulator of alternative splicing, known for causing neurodegeneration, and with complex roles in oncogenesis. We used the cluster...
Abstract Mutations in the Fused in Sarcoma (FUS) gene have been identified in familial ALS in human. Drosophila contains a single ortholog of human FUS called Cabeza (Caz). We previously established Drosophila models of ALS targeted to Caz, which developed the locomotive dysfunction and caused anatomical defects in presynaptic terminals of motoneurons. Accumulating evidence suggests that ALS and cancer share defects in many cellular processes. The Hippo pathway was originally discovered in Drosophila and plays a role as a tumor suppressor in mammals. We aimed to determine whether Hippo pathway genes modify the ALS...
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by an insufficient level of survival motor neuron (SMN) protein. It is characterized by progressive degeneration of lower motor neurons in the spinal cord, resulting in skeletal muscle atrophy and muscle weakness .