Neuromuscular Junction Acetylcholinesterase Deficiency Responsive to Albuterol
نویسندگان
چکیده
منابع مشابه
Protein-anchoring strategy for delivering acetylcholinesterase to the neuromuscular junction.
Acetylcholinesterase (AChE) at the neuromuscular junction (NMJ) is anchored to the synaptic basal lamina via a triple helical collagen Q (ColQ). Congenital defects of ColQ cause endplate AChE deficiency and myasthenic syndrome. A single intravenous administration of adeno-associated virus serotype 8 (AAV8)-COLQ to Colq(-/-) mice recovered motor functions, synaptic transmission, as well as the m...
متن کاملLocalization of "non-extractable" acetylcholinesterase to the vertebrate neuromuscular junction.
Asymmetric forms of acetylcholinesterase (AChE) are thought to be the predominant forms of this enzyme at vertebrate neuromuscular junctions where they attach to the synaptic basal lamina via a collagen-like tail. High salt and heparin-containing buffers are capable of solubilizing asymmetric AChE molecules from skeletal muscle; however, detachment of AChE specifically from synaptic basal lamin...
متن کاملOutcome of acetylcholinesterase deficiency for neuromuscular functioning.
Acetylcholinesterase (AChE) plays an essential role in neuromuscular transmission, therefore it is surprising that AChE knockout (KO) mice could live to the adulthood. Neuromuscular functioning in KO and normal (wild type, WT) mice were studied, at different age (1.5-, 4- and 9-month-old). Hindlimb muscle force productions in response to nerve or muscle electric stimulation were recorded in sit...
متن کاملIn vivo regulation of acetylcholinesterase insertion at the neuromuscular junction.
The efficiency of synaptic transmission between nerve and muscle depends on the number and density of acetylcholinesterase molecules (AChE) at the neuromuscular junction. However, little is known about the way this density is maintained and regulated in vivo. By using time lapse and quantitative fluorescence imaging assays in living mice, we demonstrated that insertion of new AChEs occurs withi...
متن کاملAcetylcholinesterase dynamics at the neuromuscular junction of live animals.
At cholinergic synapses, acetylcholinesterase (AChE) is critical for ensuring normal synaptic transmission. However, little is known about how this enzyme is maintained and regulated in vivo. In this work, we demonstrate that the dissociation of fluorescently-tagged fasciculin 2 (a specific and selective peptide inhibitor of AChE) from AChE is extremely slow. This fluorescent probe was used to ...
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ژورنال
عنوان ژورنال: Pediatric Neurology
سال: 2012
ISSN: 0887-8994
DOI: 10.1016/j.pediatrneurol.2012.04.022