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PLEKHG5抗体

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产品名称: PLEKHG5抗体
产品型号:
产品展商: XYbscience
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简单介绍

PLEKHG5抗体该基因编码的蛋白激活核因子-κB(NFKB1)信号通路。该基因突变与常染色体隐性遗传的远端脊肌萎缩症有关。已发现该基因的多个转录变体编码不同的亚型。PLEKHG5抗体[由RefSeq提供,2012年5月]


PLEKHG5抗体  的详细介绍

PLEKHG5抗体特异性结合抗原:抗体本身不能直接溶解或杀伤带有特异抗原的靶细胞,通常需要补体或吞噬细胞等共同发挥效应以**病原微生物或导致病理损伤。然而,抗体可通过与病毒或**的特异性结合,直接发挥中和病毒的作用。

产品编号xy- 12455R

英文名称PLEKHG5

中文名称凋亡诱导受体PLEKHG5抗体

别    名PKHG5_HUMAN; Pleckstrin homology domain-containing family G member 5; PH domain-containing family G member 5; Guanine nucleotide exchange factor 720; GEF720.  

说 明 书100ul  200ul

研究领域染色质和核信号  信号转导  G蛋白偶联受体  G蛋白信号  

抗体来源Rabbit

克隆类型Polyclonal

PLEKHG5抗体交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,

产品应用ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.

分 子 量116kDa

细胞定位细胞浆

性    状Lyophilized or Liquid

浓    度1mg/1ml

免 疫 原KLH conjugated synthetic peptide derived from human PLEKHG5:951-1062/1062

亚    型IgG

纯化方法affinity purified by Protein A

储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

PLEKHG5抗体保存条件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.

PubMedPubMed

产品介绍background:

This gene encodes a protein that activates the nuclear factor kappa B (NFKB1) signaling pathway. Mutations in this gene are associated with autosomal recessive distal spinal muscular atrophy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2012]


Function:

Guanine nucleotide exchange factor that activates RHOA and maybe the NF-kappa-B signaling pathway. Involved in the control of neuronal cell differentiation. Plays a role in angiogenesis through regulation of endothelial cells chemotaxis.


Subunit:

Interacts with GIPC1/synectin and RHOA.


Subcellular Location:

Cytoplasm. Cytoplasm, perinuclear region. Cell junction. Cell projection, lamellipodium. Note=Predominantly cytoplasmic, however when cells are stimulated found in perinuclear regions. Localized at cell-cell junctions in quiescent endothelial cells, it relocalizes to cytoplasmic vesicle and the leading edge of lamellipodia in migrating endothelial cells.


Tissue Specificity:

Predominantly expressed in the peripheral nervous system and brain. Highest expression is observed in heart, lung, kidney, testis and moderate expression is present in spleen, pancreas, skeletal muscle, ovary and liver. Weakly expressed in glioblastoma (GBM) cell lines.


DISEASE:

Distal spinal muscular atrophy, autosomal recessive, 4 (DSMA4) [MIM:611067]: A neuromuscular disorder. Distal spinal muscular atrophy, also known as distal hereditary motor neuronopathy, represents a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. DSMA4 is characterized by childhood onset, generalized muscle weakness and atrophy with denervation and normal sensation. Bulbar symptoms and pyramidal signs are absent. Note=The disease is caused by mutations affecting the gene represented in this entry.

Charcot-Marie-Tooth disease, recessive, intermediate type, C (CMTRIC) [MIM:615376]: A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Recessive intermediate forms of Charcot-Marie-Tooth disease are characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec. Note=The disease is caused by mutations affecting the gene represented in this entry.


Similarity:

Contains 1 DH (DBL-homology) domain.

Contains 1 PH domain.


Gene ID:

57449


PLEKHG5抗体antibody, Ab)是由效应B细胞(效应**B细胞)分泌,机体用于抵御外来物质,如病毒,**等抗原,结构呈“Y”字型的球状蛋白质,仅仅存在于脊椎动物的血液和B**细胞膜表面。凡是能够跟抗体结合的物质,均被称作抗原,因此对于抗抗体(能够结合抗体的抗体)来说,抗体本身也是一种抗原物质。

   QQ图片20171030091318

PLEKHG5抗体普通抗体重链和轻链的结构

重链结构:普通的**球蛋白具有2条重链(H链),分子量约为50kD,有μ、δ、γ、ε和α五种重链亚型,对应的**球蛋白名称分别为IgMIgGIgAIgDIgE

轻链结构:  普通**球蛋白具有2条轻链(L链),分子质量约25kDa,有κ链和λ链两种亚型,这两种轻链决定了Ig的亚型类别(IgG1IgG2IgG3IgG4)。一个天然的Ig分子两条轻链总是相同的,但在同一个体内可存在分别带有κ或λ链的抗体分子。不同种属生物体内两型轻链的比例不同,正常人血清**球蛋白κ链:λ链约为21,而在小鼠的比例为201

2.2抗体Fab段和Fc

IgG经木瓜蛋白酶酶切后裂解为2个完全相同的Fab段和1Fc,每个Fab段都为单价,可与抗原结合但不会再发生凝集反应;经胃蛋白酶酶切后裂解为1个完整F(ab)2片段和碎片化的Fc片段,F(ab)2片段为双价,可同时结合两个抗原表位。Fab段为抗原结合片段(fragment of antigen bindingFab),相当于抗体分子的两个臂,由一个完整的轻链和重链的VHCH1结构域组成。Fc段为可结晶段(fragment crystallizableFc)相当于IgCH2CH3结构域,是Ig与效应分子或者细胞相互作用的部位。Fab段包含完整的可变区,以及恒定区的CH1区域。Fc段仅指Ig恒定区CH2CH3的区域,相当于Y字结构下面那一部分。

合格 合格 AIF 调亡诱导因子抗体
合格 GLP-1 (7-36) 胰高血糖素样肽-1抗体
合格 合格 DFFB Caspase 激活的脱氧核糖核酸酶抗体
合格 BNP 脑钠素/利钠肽抗体 
合格 合格 Insulin Receptor alpha 胰岛素受体α抗体
合格 合格 Caspase-9 活化半胱胺酸蛋白酶蛋白-9抗体
合格 合格 合格 Caspase-9 活化半胱胺酸蛋白酶蛋白-9抗体
合格 Caspase 8 半胱氨酸蛋白酶8抗体
合格 Amylin 糖尿病相关肽/胰岛淀粉样肽抗体
合格 合格 CEA 癌胚抗原抗体
合格 合格 beta-Actin (Loading Control) β-肌动蛋白/β-Actin 抗体(内参抗体)
合格 Neurokinin 1 Receptor v
合格 Substance P P物质抗体
合格 合格 TGF alpha 转移生长因子α抗体
合格 NGFB 神经生长因子β抗体
合格 NFkB Inducing Kinase NIK NFkB诱导激酶抗体
合格 beta-Amyloid 1-42 (CT) β淀粉样肽1-42(C端)/β-Amyloid 1-42 (CT)抗体
合格 合格 beta-Amyloid 1-42(CT) β淀粉样肽/Aβ42抗体 
合格 合格 CD19 CD19抗体
合格 CD20 CD20抗体
合格 合格 合格 Caspase-3 活化半胱胺酸蛋白酶蛋白-3抗体
合格 Caspase-6 subunit p18 半胱胺酸蛋白酶蛋白-6抗体
合格 合格 合格 TGF beta 1 转化生长因子β1/TGF β1/TGF-β1抗体
合格 合格 caspase-3 p12 subunit 活化半胱胺酸蛋白酶蛋白3抗体
合格 Secretin 分泌素抗体
合格 Caspase-10 半胱胺酸蛋白酶-10抗体
 


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