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Rabbit Anti-MUSK Polyclonal Antibody#abs136560

Description

Catalog-specification	Delivery time	USD price
abs136560-50ug	1-2 Weeks	201.0
abs136560-100ug	1-2 Weeks	301.0

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Overview
catalog	abs136560
Description	The muscle-specific tyrosine kinase receptor is encoded by this particular gene. This receptor may have an essential role in gathering the acetylcholine receptor in the postsynaptic neuromuscular junction. Congenital myasthenic syndrome has been linked to mutations in this particular gene. There are a number of alternatively spliced transcript variants that have been reported concerning this gene.
Other names	The gene known as MuSK produces a receptor tyrosine kinase that is intimately associated with muscle development and function. This kinase is also known as the muscle-associated receptor tyrosine kinase, the muscle-specific tyrosine kinase receptor, or the skeletal muscle receptor tyrosine kinase, among other names. It plays a critical role in transmitting nerve impulses to muscle cells, enabling them to contract and generate movement. The MuSK gene is sometimes referred to by alternative designations such as CMS9, FADS, MDK4, or MGC126323. Additionally, it has been identified as neural fold somite kinase 1 (Nsk1), neural fold somite kinase 2 (Nsk2), or neural fold somite kinase 3 (Nsk3). Regardless of the specific name used, this gene is essential for proper muscle development and function. Through its role in transmitting nerve impulses to muscle cells, the MuSK kinase helps to promote the formation and maintenance of neuromuscular junctions. This complex of proteins allows nerve cells to communicate with muscle cells, coordinating their contraction and thus enabling movement. Disruptions to the MuSK gene or its protein product can lead to muscle weakness or paralysis, as seen in various neuromuscular disorders. Therefore, understanding the role of MuSK and its related kinases is critical for developing new therapies to treat these conditions.
Source	Rabbit
Specificity	Total MUSK levels can be detected by the MUSK Antibody, which specifically identifies endogenous MUSK proteins.
Species Reactivity	Human;Mouse;Rat
Antigen	MUSK
Application	For Western blot analysis, the recommended dilution range is 1:500 to 1:2000. When performing immunohistochemistry, the suggested dilution is 1:50 to 1:200. For ELISA using a peptide substrate, it is advised to dilute the samples from 1:20000 to 1:40000.
Immunogen	A synthesized peptide derived from human MUSK.
MW	97kDa
Properties
Concentration	1mg/ml
purification	The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin .
Clonality	Polyclonal Antibody
Stability & Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles
Storage buffer	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt.
Target
Background	Receptor tyrosine kinase which plays a central role in the formation and the maintenance of the neuromuscular junction (NMJ), the synapse between the motor neuron and the skeletal muscle (PubMed:25537362). Recruitment of AGRIN by LRP4 to the MUSK signaling complex induces phosphorylation and activation of MUSK, the kinase of the complex. The activation of MUSK in myotubes regulates the formation of NMJs through the regulation of different processes including the specific expression of genes in subsynaptic nuclei, the reorganization of the actin cytoskeleton and the clustering of the acetylcholine receptors (AChR) in the postsynaptic membrane. May regulate AChR phosphorylation and clustering through activation of ABL1 and Src family kinases which in turn regulate MUSK. DVL1 and PAK1 that form a ternary complex with MUSK are also important for MUSK-dependent regulation of AChR clustering. May positively regulate Rho family GTPases through FNTA. Mediates the phosphorylation of FNTA which promotes prenylation, recruitment to membranes and activation of RAC1 a regulator of the actin cytoskeleton and of gene expression. Other effectors of the MUSK signaling include DNAJA3 which functions downstream of MUSK. May also play a role within the central nervous system by mediating cholinergic responses, synaptic plasticity and memory formation (By similarity).
Posttranslational modification	Ubiquitinated by PDZRN3. Ubiquitination promotes endocytosis and lysosomal degradation (By similarity).Phosphorylated. Phosphorylation is induced by AGRIN in a LRP4-dependent manner (By similarity). Autophosphorylated (PubMed:25029443). Autophosphorylation at Tyr-554 is required for interaction with DOK7 which in turn stimulates the phosphorylation and the activation of MUSK (By similarity).Neddylated.
Celluar localization	Plasma Membrane;
UniPort	O15146