Mouse Monoclonal MuSK antibody [10A4] (STJ98259)

$379.00

Reactivity: Human
Applications: IHC, IF, ELISA
Conjugation: Unconjugated
Supplier: St John’s Laboratory Ltd.

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Mouse Monoclonal MuSK antibody [10A4] (STJ98259)

Supplier: St John’s Laboratory Ltd.

Recommended applications: IHC, IF, ELISA

Recommended dilution: IHC 1:200-1:1000; IF 1:200-1:1000; ELISA 1:10000

Recommended protocols: check protocols

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Alternative names:

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MUSK antibody,|MDK 4 antibody|MDK4 antibody|MGC126323 antibody|MGC126324 antibody|Muscle antibody|Muscle skeletal receptor tyrosine kinase antibody|Muscle skeletal receptor tyrosine protein kinase antibody|Muscle specific kinase receptor antibody|Muscle specific tyrosine kinase receptor antibody|Muscle specific tyrosine protein kinase receptor antibody|Muscle-specific kinase receptor antibody|Muscle-specific tyrosine-protein kinase receptor antibody|MuSK antibody|MUSK_HUMAN antibody|Neural fold somite kinase 1 antibody|Neural fold somite kinase 2 antibody|Neural fold somite kinase 3 antibody|Neural fold somite kinase1 antibody|Neural fold somite kinase2 antibody|Neural fold somite kinase3 antibody|Nsk 1 antibody|Nsk 2 antibody|Nsk 3 antibody|Nsk1 antibody|Nsk2 antibody|Nsk3 antibody|Receptor tyrosine kinase MuSK antibody|Skeletal muscle receptor tyrosine kinase antibody|skeletal receptor tyrosine-protein kinase antibody|Anti-MUSK antibody (ab5510)
SCBT cat No: sc-134398|sc-6009|sc-33204|sc-6010|

 

Name

MuSK Monoclonal Antibody

Catalogue No.

STJ98259

Reactivity

Human

Specificity

MuSK Monoclonal Antibody detects endogenous levels of MuSK protein.

Immunogen

Purified recombinant extracellular fragment of human MuSK (aa24-209) fused with hIgGFc tag expressed in HEK293 cell line

Host

Mouse

Applications

IHC, IF, ELISA

Recommended dilution

IHC 1:200-1:1000; IF 1:200-1:1000; ELISA 1:10000

Clonality

Monoclonal

Conjugation

Unconjugated

Isotype

IgG1

Formulation

MuSK Antibody was tube-contained. Ascitic fluid containing 0.03% sodium azide.

Concentration

1 mg/ml

Purification

MuSK Antibody was purified using affinity purification.

Storage

-20 Celsius degree. Avoid repeated freeze/thaw cycles.

Alternative antibody names

Muscle, skeletal receptor tyrosine-protein kinase antibody, Muscle-specific tyrosine-protein kinase receptor antibody, MuSK antibody, Muscle-specific kinase receptor antibody

Database links

Human UniProt/Swiss-Prot:O15146;Mouse UniPort/Swiss-Prot: Q61006;Rat UniProt/Swiss-Port: Q62838;Human Entrez Gene: 4593;Mouse Entrez Gene: 18198;Rat Entrez Gene: Rn.10210

Protein names

Muscle, skeletal receptor tyrosine-protein kinase , Muscle-specific tyrosine-protein kinase receptor , MuSK , Muscle-specific kinase receptor

Protein function

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 . 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). / ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate. / Mg2+ / Positively regulated by CK2.

Involvement in disease

Myasthenic syndrome, congenital, 9, associated with acetylcholine receptor deficiency (CMS9) [MIM:616325]: A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. CMS9 is a disorder of postsynaptic neuromuscular transmission, due to deficiency of AChR at the endplate that results in low amplitude of the miniature endplate potential and current. . Note: The disease is caused by mutations affecting the gene represented in this entry. MUSK mutations lead to decreased agrin-dependent AChR aggregation, a critical step in the formation of the neuromuscular junction.; Fetal akinesia deformation sequence (FADS) [MIM:208150]: A clinically and genetically heterogeneous group of disorders with congenital malformations related to impaired fetal movement. Clinical features include fetal akinesia, intrauterine growth retardation, polyhydramnios, arthrogryposis, pulmonary hypoplasia, craniofacial abnormalities, and cryptorchidism. . Note: The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

Belongs to the protein kinase superfamily. Tyr protein kinase family. / Contains 1 FZ (frizzled) domain. / Contains 3 Ig-like C2-type (immunoglobulin-like) domains. / Contains 1 protein kinase domain.

Protein post-translational modifications

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 . Autophosphorylation at Tyr-554 is required for interaction with DOK7 which in turn stimulates the phosphorylation and the activation of MUSK (By similarity). / Neddylated.

Protein cellular localization

Cell junction > synapse > postsynaptic cell membrane / Single-pass type I membrane protein

Research area

All research areas>Membrane Receptors>MuSK
(View all antibody categories related to Membrane Receptors)

Note

AntibodyPlus can customize MuSK Antibody according to your requirement, including bulk product size,etc. Please contact info@antibodyplus.com. AntibodyPlus provide antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

St John’s Laboratory Ltd.

Product type

Primary antibody

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