ITM0457
ITM0457
- Catalog: ITM0457
- Gene/Protein: MUSK
- Product Description: Immunotag™ MuSK Monoclonal Antibody
504.0000
Price in reward points: 504
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Antibody Specification | |
Datasheet | |
Target Protein | MuSK |
Clonality | Monoclonal |
Storage/Stability | -20°C/1 year |
Application | IHC-p,IF,ELISA |
Recommended Dilution | Immunohistochemistry: 1/200 - 1/1000. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human |
Host Species | Mouse |
Immunogen | Purified recombinant extracellular fragment of human MuSK (aa24-209) fused with hIgGFc tag expressed in HEK293 cell line. |
Specificity | MuSK Monoclonal Antibody detects endogenous levels of MuSK protein. |
Purification | Affinity purification |
Form | Ascitic fluid containing 0.03% sodium azide. |
Gene Name | MUSK |
Accession No. | O15146 Q61006 |
Alternate Names | MUSK; Muscle; skeletal receptor tyrosine-protein kinase; Muscle-specific tyrosine-protein kinase receptor; MuSK; Muscle-specific kinase receptor |
Description | muscle associated receptor tyrosine kinase(MUSK) Homo sapiens This gene encodes a muscle-specific tyrosine kinase receptor. The encoded protein may play a role in clustering of the acetylcholine receptor in the postsynaptic neuromuscular junction. Mutations in this gene have been associated with congenital myasthenic syndrome. Alternatively spliced transcript variants have been described.[provided by RefSeq, Oct 2009], |
Subcellular Localization | integral component of plasma membrane,integral component of membrane,cell junction,neuromuscular junction,receptor complex,postsynaptic membrane, |
Protein Function | catalytic activity:ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate.,disease:Defects in MUSK is a cause of autosomal recessive congenital myasthenic syndrome (CMS) [MIM:608931]. Congenital myasthenic syndromes are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. MUSK mutations lead to decreased agrin-dependent AChR aggregation, a critical step in the formation of the neuromuscular junction.,function:Receptor tyrosine kinase that is a key mediator of agrin's action and is involved in neuromuscular junction (NMJ) organization.,online information:MuSK entry,similarity:Belongs to the protein kinase superfamily. Tyr protein kinase family.,similarity:Contains 1 FZ (frizzled) domain.,similarity:Contains 1 protein kinase domain.,similarity:Contains 3 Ig-like C2-type (immunoglobulin-like) domains.,subunit:Interacts with DOK7, which probably regulates its activity., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |