产品名称
MUSK Rabbit Polyclonal Antibody
别名
Muscle, skeletal receptor tyrosine-protein kinase (EC 2.7.10.1;Muscle-specific tyrosine-protein kinase receptor;MuSK;Muscle-specific kinase receptor)
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/4593
Human Swissprot No.
O15146
Human Swissprot Link
https://www.uniprot.org/uniprotkb/O15146/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/18198
Mouse Swissprot No.
Q61006
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q61006
Rat Gene Link
https://www.uniprot.org/uniprot/81725
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/Q62838
免疫原
Synthesized peptide derived from human MUSK
特异性
This antibody detects endogenous levels of Human MUSK
稀释度
WB 1:500-2000;IHC-p 1:50-300
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
细胞定位
Cell junction, synapse, postsynaptic cell membrane ; Single-pass type I membrane protein . Colocalizes with acetylcholine receptors (AChR) to the postsynaptic cell membrane of the neuromuscular junction. .
功能
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
