接头蛋白DOK7抗体
规格:1mg/1ml
英文名: DOK7
别名: Docking protein 7; DOK 7; DOK7; DOK7_HUMAN; Downstream of tyrosine kinase 7; Protein Dok-7.
分子量: 53kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human DOK7
交叉反应:Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
细胞定位:细胞膜
接头蛋白DOK7抗体产品介绍:background: The downstream of kinase family (Dok1-7) are members of a class of “docking” proteins that include the tyrosine kinase substrates IRS-1 and Cas, which contain multiple tyrosine residues and putative SH2 binding sites. Based on their similarities, the Dok family of proteins can be divided into three subgroups: Dok-1/2/3, Dok-4/5/6 and Dok-7. Through its interaction with muscle-specific receptor kinase (MuSK), Dok-7 is crucial for neuromuscular synaptogenesis and for MuSK activation. Mice lacking Dok-7 do not form neuromuscular synapses nor acetylcholine receptor clusters. Mutations in the Dok-7 gene can cause congenital myasthenic syndromes (CMA) — 接头蛋白DOK7抗体recessively inherited disorders characterized by muscle weakness. Function: Probable muscle-intrinsic activator of MUSK that plays an essential role in neuromuscular synaptogenesis. Acts in aneural activation of MUSK and subsequent acetylcholine receptor (AchR) clustering in myotubes. Induces autophosphorylation of MUSK. Subcellular Location: Cell membrane. Cell junction > synapse. Accumulates at neuromuscular junctions. Tissue Specificity: Preferentially expressed in skeletal muscle and heart Present in thigh muscle, diaphragm and heart but not in the liver or 接头蛋白DOK7抗体spleen (at protein level). DISEASE: Defects in DOK7 are the cause of familial limb-girdle myasthenia autosomal recessive (LGM) [MIM:254300]; also called congenital myasthenic syndrome type 1B or CMS1B. LGM is a congenital myasthenic syndrome characterized by a typical 'limb girdle' pattern of muscle weakness with small, simplified neuromuscular junctions but normal acetylcholine receptor and acetylcholinesterase function. Similarity: Contains 1 IRS-type PTB domain. Contains 1 PH domain. Gene ID: 285489 Database links: Entrez Gene: 285489 Human Entrez Gene: 231134 Mouse Omim: 610285 Human SwissProt: Q18PE1 Human SwissProt: Q18PE0 Mouse Unigene: 122110 Human Unigene: 19295 Human Unigene: 701584 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
接头蛋白DOK7抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:细胞生物 神经生物学 信号转导 激酶和磷酸酶
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid