Targeting the Neuromuscular Junction in ALS
نویسندگان
چکیده
منابع مشابه
Targeting Therapy to The Neuromuscular Junction: Proof of Concept
INTRODUCTION The site of pathology in myasthenia gravis (MG) is the neuromuscular junction (NMJ). Our goal was to determine the ability to direct complement inhibition to the NMJ. METHODS A single-chain antibody directed against the alpha subunit of the acetylcholine receptor was synthesized (scFv-35) and coupled to decay-accelerating factor (DAF, scFv-35-DAF). scFv-35-DAF was tested in a pas...
متن کاملEarly and persistent abnormal decoding by glial cells at the neuromuscular junction in an ALS model.
Amyotrophic lateral sclerosis (ALS) is a late-onset neuromuscular disease characterized by progressive loss of motor neurons (MNs) preceded by neuromuscular junction (NMJ) denervation. Despite the importance of NMJ denervation in ALS, the mechanisms involved remain unexplored and ill defined. The contribution of glial cells in the disease has been highlighted, including axonal Schwann cell acti...
متن کاملThe neuromuscular junction disorders.
Neuromuscular junction (NMJ) disorders result from destruction, malfunction or absence of one or more key proteins involved in neuromuscular transmission, illustrated diagrammatically in fig 1. The most common pathology is antibody mediated damage or down regulation of ion channels or receptors, resulting in myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and acquired neuromyo...
متن کاملThe ALS gene FUS regulates synaptic transmission at the Drosophila neuromuscular junction.
Mutations in the RNA binding protein Fused in sarcoma (FUS) are estimated to account for 5-10% of all inherited cases of amyotrophic lateral sclerosis (ALS), but the function of FUS in motor neurons is poorly understood. Here, we investigate the early functional consequences of overexpressing wild-type or ALS-associated mutant FUS proteins in Drosophila motor neurons, and compare them to phenot...
متن کاملThe Neuromuscular Junction
If such a nerve fiberwere to be directly joined to amuscle fiber of, say, 50μmin diameter, then as the propagating action potential reached the nerve–muscle junction, the local circuit current density set up in the muscle would be considerably reduced from that in the nerve. This reduction would occur because the nerve and muscle must have the same total current, a consequence of current conser...
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ژورنال
عنوان ژورنال: Neurotherapeutics
سال: 2018
ISSN: 1933-7213,1878-7479
DOI: 10.1007/s13311-018-0647-y