97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
字幕av,91久久精品一二三区,四虎高清视频
首頁 > 產品中心 > 標記一抗 > 產品信息
Mouse Anti-MUSK/BF350 Conjugated antibody (bsm-51427M-BF350)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bsm-51427M-BF350
英文名稱 Mouse Anti-MUSK/BF350 Conjugated antibody
中文名稱 BF350標記的小鼠抗肌肉骨骼受體酪氨酸激酶單克隆抗體
別    名 skeletal receptor tyrosine-protein kinase; MDK 4; MDK4; Muscle; Muscle skeletal receptor tyrosine kinase; Muscle skeletal receptor tyrosine protein kinase; Muscle specific kinase receptor; Muscle specific tyrosine kinase receptor; Muscle specific tyrosine protein kinase receptor; Muscle-specific kinase receptor; Muscle-specific tyrosine-protein kinase receptor; MuSK; Neural fold somite kinase 1; Neural fold somite kinase 2; Neural fold somite kinase 3; Neural fold somite kinase1; Neural fold somite kinase2; Neural fold somite kinase3; Nsk 1; Nsk 2; Nsk 3; Nsk1; Nsk2; Nsk3; Nsk-1; Nsk-2; Nsk-3; Receptor tyrosine kinase MuSK; Skeletal muscle receptor tyrosine kinase; MUSK_MOUSE.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  信號轉導  激酶和磷酸酶  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 6D1
交叉反應 (predicted: Mouse, Rat, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 97kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 Recombinant mouse MUSK protein
亞    型 IgG1
純化方法 affinity purified by Protein G
儲 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
Receptor tyrosine kinases (RTKs) represent an important class of transmembrane signaling molecules. Binding of the extracellular domain of an RTK to its cognate ligand leads to receptor dimerization and the activation of the intrinsic tyrosine kinase activity of its intracellular kinase domain. The specificity of this type of cellular communication is conferred in part by the distribution of the receptor, which determines the cells that are capable of responding to a given ligand. MuSK, for muscle-specific kinase, is an RTK that is uniquely specific to the skeletal muscle lineage. MuSK is expressed at low levels in proliferating myoblasts, but is induced upon terminal differentiation and myotube fusion. In the embryo, MuSK is expressed in developing muscle, but its level of expression is dramatically reduced in mature muscle, where it is abundant only at the neuromuscular junction. The human MuSK gene maps to chromosome 9q31.3, overlapping a region containing the Fukuyama muscular dystrophy mutation.

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

Subunit:
Monomer (By similarity). Homodimer (Probable). Interacts with LRP4; the heterodimer forms an AGRIN receptor complex that binds AGRIN resulting in activation of MUSK (By similarity). Forms a heterotetramer composed of 2 DOK7 and 2 MUSK molecules which facilitates MUSK trans-autophosphorylation on tyrosine residue and activation. Interacts (via cytoplasmic part) with DOK7 (via IRS-type PTB domain); requires MUSK phosphorylation. Interacts with DVL1 (via DEP domain); the interaction is direct and mediates the formation of a DVL1, MUSK and PAK1 ternary complex involved in AChR clustering (By similarity). Interacts with PDZRN3; this interaction is enhanced by agrin (By similarity). Interacts with FNTA; the interaction is direct and mediates AGRIN-induced phosphorylation and activation of FNTA (By similarity). Interacts with CSNK2B; mediates regulation by CK2 (By similarity). Interacts (via the cytoplasmic domain) with DNAJA3 (By similarity). Interacts with NSF; may regulate MUSK endocytosis and activity (By similarity). Interacts with CAV3; may regulate MUSK signaling (By similarity). Interacts with RNF31 (By similarity).

Subcellular Location:
Cell junction, synapse, postsynaptic cell membrane; Single-pass type I membrane protein (Probable). Note=Localizes to the postsynaptic cell membrane of the neuromuscular junction

Post-translational modifications:
Ubiquitinated by PDZRN3. Ubiquitination promotes endocytosis and lysosomal degradation (By similarity).
Phosphorylated. Phosphorylation is induced by AGRIN. Autophosphorylation at Tyr-554 is required for interaction with DOK7 which in turn stimulates the phosphorylation and the activation of MUSK.

DISEASE:
Defects in MUSK is a cause of congenital myasthenic syndrome with acetylcholine receptor deficiency (CMS-ACHRD) [MIM:608931]. A postsynaptic congenital myasthenic syndrome. Mutations underlying AChR deficiency cause a 'loss of function' and show recessive inheritance. Note=MUSK mutations lead to decreased agrin-dependent AChR aggregation, a critical step in the formation of the neuromuscular junction.

Similarity:
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.

Database links:

Entrez Gene: 4593 Human

Entrez Gene: 18198 Mouse

Entrez Gene: 81725 Rat

Omim: 601296 Human

SwissProt: O15146 Human

SwissProt: Q61006 Mouse

SwissProt: Q62838 Rat

Unigene: 521653 Human

Unigene: 16148 Mouse

Unigene: 10210 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 日韩城人免费 | 日本牲交大片免费观看 | 成在人线AV无码免费高潮喷水 | 日本xxxx裸体xxxx野外 | 成人亚洲A片V一区二区三区色欲 | www.久草视频 | 天天做天天爱天天综合网2021 | 久操资源 | 国产午夜日韩不卡一区 | 一边捏奶头一边高潮视频 | 在线A亚洲老鸭窝天堂AV高清 | 国产黑色丝袜在线观看下 | 国产成人自拍视频在线 | 99久久精品免费观看国产 | 日韩一级黄色大片 | 亚洲中文字幕久久精品无码va | 澳门黄色网 | 日韩欧美一区二区三区四区 | 在线视频观看一区二区 | 97在线观看免费版高清 | 免费一级好看的国产 | 麻豆安全免费网址入口 | 草久在线播放 | 一级全黄男女免费大片 | 五月丁香六月综合AV | 人人干日日操 | 亚洲精品日本无v一区 | 久操国产精品 | 日韩av高清在线 | 日本熟妇色xxxxx | 插插插干干干 | 国产伦精品一区二区三区网站 | 久久婷婷日日澡天天添 | 亚洲三级高清日本 | 日本三级吃奶头添泬 | 伊人网络 | ASSFREE疯狂老妇熟女 | 亚洲第一网站 | 伊甸园精品区 | 激情婷婷 | 国产三级午夜理伦三级最新章节 |